BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of ...BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of specific clinical manifestations and imaging features,UPS diagnosis mainly depends on pathological and immunohistochemical examinations for exclusive diagnosis.Here we report an extremely rare case of high-grade UPS in the common bile duct(CBD).There are limited available data on such cases.CASE SUMMARY A 70-year-old woman was admitted to our department with yellow eyes and urine accompanied by upper abdominal distending pain for 2 wk.Her laboratory data suggested significantly elevated hepatorenal function levels.The imaging data revealed calculous cholecystitis,intrahepatic and extrahepatic bile duct dilation with extrahepatic bile duct calculi,and a space-occupying lesion at the distal CBD.After endoscopic biliary stenting and symptomatic support therapy,CBD exploration and biopsy were performed.The frozen section indicated malignant spindle cell tumor of the CBD mass,and further radical pancreaticoduodenectomy was performed.Finally,the neoplasm was diagnosed as a high-grade UPS combined with the light-microscopic morphology and immunohistochemical results.CONCLUSION This extremely rare case highlighted the need for increasing physicians'vigilance,reducing the odds of misdiagnosis,and providing appropriate treatment strategies.展开更多
BACKGROUND Rhabdomyosarcoma(RMS)of the vagina in postmenopausal women is an extre-mely rare malignant tumor that was originally described as a unique group of soft tissue sarcomas originating from primitive mesenchyma...BACKGROUND Rhabdomyosarcoma(RMS)of the vagina in postmenopausal women is an extre-mely rare malignant tumor that was originally described as a unique group of soft tissue sarcomas originating from primitive mesenchymal cells.It was first re-ported in postmenopausal women in 1970,and fewer than 50 postmenopausal patients have been reported to date.CASE SUMMARY A 68-year-old multiparous female was admitted to the hospital on October 11,2023,with the chief complaint of a mass causing vaginal prolapse with incomplete urination that had persisted for 4 months.The vaginal mass was approximately the size of a pigeon egg;after lying down,the vaginal mass retracted.Complete resection was performed,and vaginal pleomorphic RMS was diagnosed based on pathology and immunohistochemical staining features.The patient is currently undergoing chemotherapy.The present study also reviewed the clinical,histolo-gical,and immunohistochemical features and latest treatment recommendations for vaginal RMS.Any abnormal vaginal mass should be promptly investigated through pelvic examination and appropriate imaging.The current initial treat-ment for vaginal RMS is biopsy and primary chemotherapy.CONCLUSION When surgery is planned for vaginal RMS,an organ-preserving approach should be considered.展开更多
BACKGROUND Salivary gland tumors are relatively rare.Most minor salivary gland tumors are malignant with benign tumors accounting for 18%of the tumors.Pleomorphic adenoma(PA)is the most common salivary gland tumor.Lip...BACKGROUND Salivary gland tumors are relatively rare.Most minor salivary gland tumors are malignant with benign tumors accounting for 18%of the tumors.Pleomorphic adenoma(PA)is the most common salivary gland tumor.Lip PA is uncommon with 9.8%occurring in the upper lip.We are adding on the knowledge of the rare upper lip PA(benign mixed tumor).CASE SUMMARY We report an upper lip PA(benign mixed tumor)in a 28-year-old man.His complaint was a painless swelling on the upper lip.A painless,non-tender,well-circumscribed,slightly mobile,sessile,nodular,and rubbery(in consistency)tumor measuring 5.0 cm x 2.0 cm was noted on the left side of his upper lip.The overlying skin was not fixed and of normal color.There was no ulceration,and palpation did not elicit pain or bleeding.There was no history of trauma.Blunt dissection was used to completely excise the nodular,whitish,and encapsulated tumor.Microscopy showed a well-circumscribed and partly encapsulated biphasic lesion,with large lobules of myxo-chondroid stroma and intervening cellular nodules of basaloid cells,well-formed tubules containing eosinic secretion,and nests of myoepithelial cells.A diagnosis of PA(benign mixed tumor)was confirmed.CONCLUSION Blunt dissection is indicated to preserve the cosmesis and function of the upper lip.展开更多
BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphi...BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine patients.CASE SUMMARY The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and liver.His most recent positron emission tomography-computed tomography(PET-CT)scan showed uptake in the ascending and transverse colons.A colonoscopy revealed a 5.0 cm×3.5 cm×3.0 cm pedunculated polyp in the ascending colon.The polyp was removed using hot snare polypectomy technique and retrieved with Rothnet.Histopathologic examination of the polyp showed a metastatic pleomorphic leiomyosarcoma.CONCLUSION Uptake(s)on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.展开更多
Background: A number of meningeal neoplastic lesions may radiologically and clinically simulate meningioma, include hemangiopericytomas, solitary fibrous tumors, schwannomas, hematolymphoid lesions, metastases, and ot...Background: A number of meningeal neoplastic lesions may radiologically and clinically simulate meningioma, include hemangiopericytomas, solitary fibrous tumors, schwannomas, hematolymphoid lesions, metastases, and others very rarely, also may clinically mimic meningiomas. Case Description: We present the case of A 28-year-old male patient, with no notable medical history, who presented with worsening headaches for 3 months, imbalance, and visual deficits, An initial MRI revealed extra-axial lesion involving the right Parieto-occipital, The tumor was hypointense on T1-weighted MR images, hyperintense signals on T2-weightedMR images, and heterogeneously enhanced suggestive of a meningioma, total resection was achieved, and the histopathological analysis confirmed the diagnosis of an angioblastic meningioma. However, 15 months later, the patient presented with the same initial visual complaints. A subsequent MRI showed lesion recurrence, leading to a second surgical intervention. The histopathological analysis confirmed the diagnosis of an anaplastic xanthoastrocytoma. Conclusion: This represents an unusual location for an anaplastic pleomorphic xanthoastrocytoma, which should broaden the differential diagnosis of extra-axial lesions.展开更多
Pleomorphic adenoma is the most common tumor of the salivary glands, accounting for approximately 50% of salivary gland lesions. It develops mainly in the salivary glands: parotid (80%), submaxillary (10%), sublingual...Pleomorphic adenoma is the most common tumor of the salivary glands, accounting for approximately 50% of salivary gland lesions. It develops mainly in the salivary glands: parotid (80%), submaxillary (10%), sublingual (1%) and in the accessory oral-pharyngeal glands (9%). The aim of this work was to report 2 cases of pleomorphic adenoma of atypical location and then to discuss the difficulties linked to its diagnostic and therapeutic management in the Malian context. They were a 40-year-old man and a 72-year-old woman. They were admitted to the ENT department of the “Luxembourg Mere-infant” hospital for oropharyngeal swelling for the first and swelling of the palate for the second. The clinical expression was a swelling in both cases, of a hard, mobile consistency with healthy mucosa on their surface. The remainder of the physical examination was unremarkable. The diagnosis of a tumor of the oropharynx and palate was made following clinical radiological examinations. The histopathological examination of the surgical specimen made it possible to make the diagnosis of pleomorphic adenoma of the palate and the left palatine tonsil. The follow-up surgery was straightforward, with a favorable outcome. Pleomorphic adenomas are relatively rare benign tumors of the accessory salivary glands;their clinical expression remains swelling. Therapeutic management is surgery and the diagnosis is confirmed by histopathological examination.展开更多
Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are one spectrum of rare cutaneous neoplasms that typically arise in sun-exposed skin of older population. AFX/PDS is essentially diagnos...Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are one spectrum of rare cutaneous neoplasms that typically arise in sun-exposed skin of older population. AFX/PDS is essentially diagnosis of exclusion requiring Immunohistochemical work-up to exclude other types of tumors. Case Report: We present a case involving an ulcerated solitary lesion on the scalp of an elderly man. Histological examination revealed that the dermal tumor was composed of large pleomorphic, epithelioid, and spindle cells with clear cytoplasm. These cells were negative for cytokeratins, melanocytes and smooth muscle markers, but positive for CD10. These findings are consistent with a diagnosis of clear cell (CC) PDS. Conclusion: PDS is a low-grade malignancy that can recur locally and metastasize, which is distinguished from AFX by its larger size and the presence of aggressive histopathologic features including deeper invasion into the subcutaneous tissue, tumor necrosis, and lymphovascular and/or perineural involvement. Among several histopathologic variants, the CC variant is extremely rare with only two cases of PDS reported in the literature to date.展开更多
·AIM: To detect proteomic differences in tears between adenoid cystic carcinoma(ACC) and pleomorphic adenoma(PA).·METHODS: Tear samples were collected from 4 patients with ACC, 5 with PA, and 4 control cases...·AIM: To detect proteomic differences in tears between adenoid cystic carcinoma(ACC) and pleomorphic adenoma(PA).·METHODS: Tear samples were collected from 4 patients with ACC, 5 with PA, and 4 control cases. Label-free analysis and parallel reaction monitoring(PRM) were used to screen and validate the tear proteome. Gene Ontology(GO) annotation and Kyoto Encyclopedia of Genes and Genomes(KEGG) were conducted for bioinformatics analysis.·RESULTS: In total, 1059 proteins in tear samples were identified by label-free analysis. Between ACC and PA, 415 differentially expressed proteins were detected. Based on the GO annotation, enzyme regulator activity and serinetype endopeptidase inhibitor activity in the molecular function category, blood microparticle and extracellular matrix in the cellular component category, and response to nutrient levels in the biological process category were most predominant. By KEGG pathway annotation, the different proteins between ACC and PA mainly par ticipated in complement and coagulation cascades, amoebiasis, African trypanosomiasis and cholesterol metabolism. Eight proteins with mostly significant differences were verified by PRM, and five proteins with more than 10-fold increases in ACC compared with PA, including integrin β, α-2-macroglobulin, epididymal secretory sperm binding protein Li 78p, RAB5C, and complement C5, were identified.·CONCLUSION: The combined tools of label-free analysis and PRM are very effective and efficient, especially for samples such as tears. Some proteomic dif ferences in tears between ACC and PA are identified and these protein candidates may be specific biomarkers for future exploration.展开更多
Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,p...Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,progresses rapidly,and is prone to remote metastasis.Currently,there is no standard protocol for the treatment of thyroid sarcomas,and most treatment effects are unsatisfactory.Argon-helium cryoablation is an important method of local treatment that is widely used in patients with unresectable advanced tumors.However,owing to the low incidence of thyroid sarcomas,there are no relevant literature reports on the treatment of thyroid sarcomas using cryoablation in China.This study reports the case of a patient with undifferentiated pleomorphic sarcoma of the thyroid gland who was treated with argon-helium cryoablation,and the immediate outcome was good.Based on a review of relevant literature,we discussed the effectiveness and safety of argon-helium cryoablation treatment,to provide clinical guidance and references for the treatment of patients with thyroid sarcoma.展开更多
Background: Mucoepidermoid carcinoma ex-pleomorphic adenoma (MECxPA) is an extremely rare salivary gland malignancy. With only nine prior reported cases, this entity represents a challenging histopathological diagnosi...Background: Mucoepidermoid carcinoma ex-pleomorphic adenoma (MECxPA) is an extremely rare salivary gland malignancy. With only nine prior reported cases, this entity represents a challenging histopathological diagnosis. Methods: We present a case of a 71-year-old male with an enlarging left neck mass over several months. CT showed both a parapharyngeal space mass and a separate level II neck mass. Results: The patient underwent resection of the left parapharyngeal mass and ipsilateral selective level II-IV lymphadenectomy. The final pathologic diagnosis was metastatic high-grade mucoepidermoid carcinoma ex-pleomorphic adenoma. Conclusions: We describe a novel presentation of high-grade mucoepidermoid carcinoma ex-pleomorphic adenoma as a metastatic parapharyngeal mass.展开更多
Giant cell tumors of the pancreas come in three varieties-osteoclastic,pleomorphic,and mixed histology.These tumors have distinctive endoscopic,clinical,and cytological features.Giant cell tumors have a controversial ...Giant cell tumors of the pancreas come in three varieties-osteoclastic,pleomorphic,and mixed histology.These tumors have distinctive endoscopic,clinical,and cytological features.Giant cell tumors have a controversial histogenesis,with some authors favoring an epithelial origin and others favoring a mesenchymal origin.The true origin of these lesions remains unclear at this time.These are also very rare tumors but proper identification and differentiation from more common pancreatic adenocarcinoma is important.The risk factors of these tumors and the prognosis may be different from those associated with standard pancreatic adenocarcinoma.Recognition of these differences can significantly affect patient care.These lesions have a unique appearance when imaged with endoscopic ultrasound(EUS),and these lesions can be diagnosed via EUS guided Fine Needle Aspiration(FNA).This manuscript will review the endoscopic,clinical,and pathologic features of these tumors.展开更多
BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been...BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been reported in the English literature. METHODS:We report a rare case of a hepatic UPS presenting as a unilocular cystic lesion with respect to histopathologic features,the newly revised diagnostic criteria,and the differential diagnosis. RESULTS:A 60-year-old man presented for evaluation of epigastric pain of 7 months duration.Abdominal computed tomography revealed a hypodense,unilocular cystic mass in the right lobe of the liver,measuring 14.0×11.1 cm.A right lobectomy was performed.Grossly,the cut surface showed a single,well-circumscribed unilocular cystic tumor mass containing dark red-brown necrotic debris and blood clots, which occupied most of the mass.Microscopically,the tumor consisted of haphazardly arranged mononuclear pleomorphic tumor cells,admixed with abundant osteoclast- like multinucleated giant cells.Immunohistochemically,the tumor cells expressed vimentin only.The histopathologic and immunohistochemical findings were compatible with a UPS.The patient is alive and well 41 months after surgery without recurrence. CONCLUSIONS:Clinically,most of the hepatic UPSs are solid masses.Only two cases have presented as multilocular cystic masses.A primary hepatic UPS presenting as a unilocular cyst has never been reported.A UPS should be included in the differential diagnosis of unilocular cystic lesions in the liver.展开更多
BACKGROUND Carcinoma ex pleomorphic adenoma (CXPA) is defined as a malignant salivary gland tumor arising from a primary or recurrent pleomorphic adenoma.Only three cases of CXPA of the trachea have been reported in t...BACKGROUND Carcinoma ex pleomorphic adenoma (CXPA) is defined as a malignant salivary gland tumor arising from a primary or recurrent pleomorphic adenoma.Only three cases of CXPA of the trachea have been reported in the literature.CASE SUMMARY We report a case of tracheal CXPA in a 55-year-old woman,who presented with a more than 3-mo history of progressive dyspnea.Computed tomography of the neck and thorax revealed an inhomogeneous,broad-based lesion arising from the tracheal wall on the right side.Endoscopy revealed a subglottic neoplasm causing up to 90% luminal stenosis.The tumor was resected using a highfrequency electrosurgical snare combined with argon plasma coagulation.Histopathology and immunohistochemistry revealed that the tumor was a CXPA of the trachea.CONCLUSION We report the fourth case of tracheal CXPA,and present the first instance of resection of CXPA using high-frequency electrosurgical snare and laser ablation.We also discuss the pathogenesis,diagnosis,histopathology,and systemic therapy of this rare disease.展开更多
AIM: To investigate C-myc, Ki-67, pan-cytokeratin, and vimentin immunohistochemical features of carcinoma ex pleomorphic adenoma(Ca-ex-PA) and pleomorphic adenoma(PA) in the lacrimal gland in order to find some clues ...AIM: To investigate C-myc, Ki-67, pan-cytokeratin, and vimentin immunohistochemical features of carcinoma ex pleomorphic adenoma(Ca-ex-PA) and pleomorphic adenoma(PA) in the lacrimal gland in order to find some clues in the differential diagnosis between them.METHODS: We reviewed microscopic slides and clinical records of 64 cases of PA and 15 cases of Ca-ex-PA in the lacrimal gland. Immunohistochemical antibodies for C-myc, Ki-67, pan-cytokeratin, and vimentin were employed.RESULTS: Median age of PA was 43.2 y(from 21 to 75). The 35 patients(54.7%) were male and 29 patients(45.3%) were female. For the PAs, the average positivity of C-myc was 4.6%;the average proliferation index of Ki-67 was 3.2%;pan-cytokeratin was positive in ductal cells, and vimentin was positive in myoepithelial cells. Median age of Ca-ex-PA was 54.3 y(from 26 to 76). There were 7 male patients(46.7%) and 8 female patients(53.3%). Among 15 Ca-ex-PAs, there were 6 myoepithelial carcinomas, 4 adenocarcinomas, 3 epithelial-myoepithelial carcinomas, and 2 squamous cell carcinomas. For the Ca-ex-PAs, the average positivity of C-myc was 36.4%;the average proliferation index of Ki-67 was 29.2%;pan-cytokeratin was positive in all cases, and vimentin was positive in myoepithelial carcinomas.CONCLUSION: PA has a lower positivity of C-myc and Ki-67, while Ca-ex-PA had a higher positivity of these two biomarkers. These four biomarkers as a set could provide valuable clues in the differential diagnosis between Ca-exPA and PA. Our results indicate that the activation of C-myc could play an important role in the pathogenesis of Ca-exPA and PA.展开更多
BACKGROUND Pleomorphic lipoma (PL) is a rare benign mesenchymal tumor occurring predominantly in middle-aged and elderly men. It is typically found in the subcutaneous tissue of the posterior neck, back, and shoulders...BACKGROUND Pleomorphic lipoma (PL) is a rare benign mesenchymal tumor occurring predominantly in middle-aged and elderly men. It is typically found in the subcutaneous tissue of the posterior neck, back, and shoulders. There have also been a few reported cases in the face, scalp, and upper extremities. Currently, there is no report on its occurrence in the anterior mediastinum. CASE SUMMARY Herein, we report the case of a 67-year-old woman diagnosed with PL in the anterior mediastinum. The tumor was removed by thoracoscopic surgery. There was no recurrence during the 24-mo follow-up period, and the prognosis was good. Most PL are located on the skin surface. However, they may also occur within the body, even in the mediastinum. CONCLUSION PL occurring in the anterior mediastinum is rare, and it may be differentiated from other tumors.展开更多
AIM:To monitor the prognosis of patients with lacrimal gland pleomorphic adenoma(LGPA)following surgical resection within the past 10y.The factors affecting patient prognosis are analyzed and the experience of surgica...AIM:To monitor the prognosis of patients with lacrimal gland pleomorphic adenoma(LGPA)following surgical resection within the past 10y.The factors affecting patient prognosis are analyzed and the experience of surgical treatment is summarized.METHODS:In this retrospective,comparative case series,clinical records from 109 cases of LGPA treated chiefly at the same institution between November 2009 and May 2019 were reviewed.All 109 patients underwent surgery,histopathological examination,and imaging examination.For patients who underwent surgery for the first time,LGPA could be resected completely,including tumor and capsule tissues,using a surgical approach via the eyebrow arch or double eyelid crease.RESULTS:The ratio of males to females was 1:1.60,the ages ranged from 19 to 74 years old with a mean age of 43.64±13.07 years old,and the ratio of left to right eyes was 1:1.37.A total of 109 patients underwent surgical excision and five of these收稿日期radiotherapy after malignant transformation.Of these patients,15 were lost to follow up within the April 1,2020 deadline and 1 was diagnosed as a recurring pathology.The 5-year recurrence rate for 86 patients who underwent initial surgery was 7.27%.Single factor analysis revealed that the course of disease,bone destruction,invasion of surrounding tissues,tumor size,capsule integrity,and expression of Ki-67 were statistically significant(P<0.05).Binomial Logistic regression analysis showed that capsule integrity was a risk factor influencing recurrence(P=0.008).CONCLUSION:LGPA has a risk of recurrence and potential for malignant transformation.Complete removal of the tumor and capsule in the initial surgery is a key factor in preventing recurrence.展开更多
BACKGROUND Pleomorphic rhabdomyosarcoma(RMS)of the spermatic cord is a group of rare neoplasms,and a secondary hydrocele testis occasionally occurs.The misdiagnosis of paratesticular mass may lead to a therapeutic del...BACKGROUND Pleomorphic rhabdomyosarcoma(RMS)of the spermatic cord is a group of rare neoplasms,and a secondary hydrocele testis occasionally occurs.The misdiagnosis of paratesticular mass may lead to a therapeutic delay.CASE SUMMARY A 79-year-old man presented to our clinic complaining of a 1-mo history of painless scrotal swelling.Physical examination revealed approximately a 15 cm×10 cm×5 cm inguinal mass with limited mobility.Contrast-enhanced magnetic resonance imaging showed a hydrocele testis,several enlarged inguinal lymph nodes,and a heterogeneously enhanced lesion with a relatively well-defined margin in the left inguinal region.Due to the imaging findings,he was diagnosed with pleomorphic RMS and received a wide resection of the mass,an inguinal incision with a high section of the left spermatic cord,and a left radical orchiectomy.He experienced local relapse 1 mo postoperatively and received radiotherapy and anlotinib hydrochloride-based immunotherapy as adjuvant therapy.The patient died 3 mo after the surgery.CONCLUSION The optimal interventions for advanced-stage pleomorphic RMS patients should be investigated by more preclinical studies and clinical trials.Physicians need to be aware of the occurrence of pleomorphic RMS in unusual locations,especially when accompanied by a hydrocele testis.展开更多
We present a case of spontaneous undifferentiated/unclassified sarcoma,of a pleomorphic subtype formerly known as malignant fibrous histiocytoma(UPS/MFH),arising from the pancreas of a laboratory rat.The mass was exci...We present a case of spontaneous undifferentiated/unclassified sarcoma,of a pleomorphic subtype formerly known as malignant fibrous histiocytoma(UPS/MFH),arising from the pancreas of a laboratory rat.The mass was excised after laparotomy from a 6‐month‐old female laboratory Wistar rat.It presented a giant multilobulated mass of irregular shape,which had arisen from the pancreas and occupied almost the entire peritoneal cavity.Histologically the tumor was characterized by a highly variable morphological pattern,with frequent transitions from storiform to pleomorphic areas.An extensive immunohistochemical examination revealed no specific lines of differentiation.Immunohistochemical positivity was observed only to MIB‐1(high Ki‐67 proliferation index),vimentin and CD68 antibodies.The diagnosis was compatible with UPS/MFH.To the best of our knowledge,the present case is the first report of a spontaneous primary UPS/MFH arising from the pancreas of a laboratory rat.展开更多
BACKGROUND Myoepithelial carcinoma(MC)is a clinically rare malignancy,there is controversy regarding its etiology and its biological behavior is not fully elucidated.Extensive surgical resection is the main treatment ...BACKGROUND Myoepithelial carcinoma(MC)is a clinically rare malignancy,there is controversy regarding its etiology and its biological behavior is not fully elucidated.Extensive surgical resection is the main treatment method.We describe a case of pleomorphic adenoma(PA)with multiple postoperative recurrences after malignant transformation,and the history of the disease in this patient was more than 20 years.Complete resection during the first surgery of PA and long-term postoperative follow-up is necessary.CASE SUMMARY A 34-year-old male with PA and a history of 5 postoperative recurrences over 21 years,each surgically removed,presented 15 d ago with headache,nasal congestion,protrusion of the right eyeball and loss of vision in the right eye,with progressively worsening symptoms.The patient underwent surgery,and MC was confirmed by pathology examination.A small PA component was locally visible under light microscope.The patient had a recurrence of the tumor 2 mo after surgery and underwent surgical resection.CONCLUSION During the first operation for PA,care should be taken not to rupture the envelope to prevent tumor cell implantation,and when complete resection is not possible due to the anatomical site,postoperative radiotherapy is necessary to control the lesion and prevent infiltration and malignant transformation of the tumor to MC.Computed tomography and magnetic resonance imaging is important for establishing diagnosis and developing a treatment plan.展开更多
We report a case of carcinoma ex pleomorphic adenoma of a sublingual gland in a 70-year-old man.Under a clinical diagnosis of benign salivary gland tumor,excision of the mass with the sublingual salivary gland in an e...We report a case of carcinoma ex pleomorphic adenoma of a sublingual gland in a 70-year-old man.Under a clinical diagnosis of benign salivary gland tumor,excision of the mass with the sublingual salivary gland in an en bloc fashion via an intraoral approach was performed.Histopathologically,there was a rupture of the fibrous capsule and diffuse cell-rich sheets composed of myoepithelial cells with round nuclei were also seen.Immunohistochemically,the cells that composed of cell rich sheets were positive to smooth muscle actin.Final diagnosis of myoepithelial carcinoma ex pleomorphic adenoma was made.展开更多
基金Supported by the People’s Livelihood Science and Technology Innovation Project of the Bureau of Science and Technology of Jiaxing City,No.2021AD30091.
文摘BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of specific clinical manifestations and imaging features,UPS diagnosis mainly depends on pathological and immunohistochemical examinations for exclusive diagnosis.Here we report an extremely rare case of high-grade UPS in the common bile duct(CBD).There are limited available data on such cases.CASE SUMMARY A 70-year-old woman was admitted to our department with yellow eyes and urine accompanied by upper abdominal distending pain for 2 wk.Her laboratory data suggested significantly elevated hepatorenal function levels.The imaging data revealed calculous cholecystitis,intrahepatic and extrahepatic bile duct dilation with extrahepatic bile duct calculi,and a space-occupying lesion at the distal CBD.After endoscopic biliary stenting and symptomatic support therapy,CBD exploration and biopsy were performed.The frozen section indicated malignant spindle cell tumor of the CBD mass,and further radical pancreaticoduodenectomy was performed.Finally,the neoplasm was diagnosed as a high-grade UPS combined with the light-microscopic morphology and immunohistochemical results.CONCLUSION This extremely rare case highlighted the need for increasing physicians'vigilance,reducing the odds of misdiagnosis,and providing appropriate treatment strategies.
文摘BACKGROUND Rhabdomyosarcoma(RMS)of the vagina in postmenopausal women is an extre-mely rare malignant tumor that was originally described as a unique group of soft tissue sarcomas originating from primitive mesenchymal cells.It was first re-ported in postmenopausal women in 1970,and fewer than 50 postmenopausal patients have been reported to date.CASE SUMMARY A 68-year-old multiparous female was admitted to the hospital on October 11,2023,with the chief complaint of a mass causing vaginal prolapse with incomplete urination that had persisted for 4 months.The vaginal mass was approximately the size of a pigeon egg;after lying down,the vaginal mass retracted.Complete resection was performed,and vaginal pleomorphic RMS was diagnosed based on pathology and immunohistochemical staining features.The patient is currently undergoing chemotherapy.The present study also reviewed the clinical,histolo-gical,and immunohistochemical features and latest treatment recommendations for vaginal RMS.Any abnormal vaginal mass should be promptly investigated through pelvic examination and appropriate imaging.The current initial treat-ment for vaginal RMS is biopsy and primary chemotherapy.CONCLUSION When surgery is planned for vaginal RMS,an organ-preserving approach should be considered.
文摘BACKGROUND Salivary gland tumors are relatively rare.Most minor salivary gland tumors are malignant with benign tumors accounting for 18%of the tumors.Pleomorphic adenoma(PA)is the most common salivary gland tumor.Lip PA is uncommon with 9.8%occurring in the upper lip.We are adding on the knowledge of the rare upper lip PA(benign mixed tumor).CASE SUMMARY We report an upper lip PA(benign mixed tumor)in a 28-year-old man.His complaint was a painless swelling on the upper lip.A painless,non-tender,well-circumscribed,slightly mobile,sessile,nodular,and rubbery(in consistency)tumor measuring 5.0 cm x 2.0 cm was noted on the left side of his upper lip.The overlying skin was not fixed and of normal color.There was no ulceration,and palpation did not elicit pain or bleeding.There was no history of trauma.Blunt dissection was used to completely excise the nodular,whitish,and encapsulated tumor.Microscopy showed a well-circumscribed and partly encapsulated biphasic lesion,with large lobules of myxo-chondroid stroma and intervening cellular nodules of basaloid cells,well-formed tubules containing eosinic secretion,and nests of myoepithelial cells.A diagnosis of PA(benign mixed tumor)was confirmed.CONCLUSION Blunt dissection is indicated to preserve the cosmesis and function of the upper lip.
文摘BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine patients.CASE SUMMARY The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and liver.His most recent positron emission tomography-computed tomography(PET-CT)scan showed uptake in the ascending and transverse colons.A colonoscopy revealed a 5.0 cm×3.5 cm×3.0 cm pedunculated polyp in the ascending colon.The polyp was removed using hot snare polypectomy technique and retrieved with Rothnet.Histopathologic examination of the polyp showed a metastatic pleomorphic leiomyosarcoma.CONCLUSION Uptake(s)on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.
文摘Background: A number of meningeal neoplastic lesions may radiologically and clinically simulate meningioma, include hemangiopericytomas, solitary fibrous tumors, schwannomas, hematolymphoid lesions, metastases, and others very rarely, also may clinically mimic meningiomas. Case Description: We present the case of A 28-year-old male patient, with no notable medical history, who presented with worsening headaches for 3 months, imbalance, and visual deficits, An initial MRI revealed extra-axial lesion involving the right Parieto-occipital, The tumor was hypointense on T1-weighted MR images, hyperintense signals on T2-weightedMR images, and heterogeneously enhanced suggestive of a meningioma, total resection was achieved, and the histopathological analysis confirmed the diagnosis of an angioblastic meningioma. However, 15 months later, the patient presented with the same initial visual complaints. A subsequent MRI showed lesion recurrence, leading to a second surgical intervention. The histopathological analysis confirmed the diagnosis of an anaplastic xanthoastrocytoma. Conclusion: This represents an unusual location for an anaplastic pleomorphic xanthoastrocytoma, which should broaden the differential diagnosis of extra-axial lesions.
文摘Pleomorphic adenoma is the most common tumor of the salivary glands, accounting for approximately 50% of salivary gland lesions. It develops mainly in the salivary glands: parotid (80%), submaxillary (10%), sublingual (1%) and in the accessory oral-pharyngeal glands (9%). The aim of this work was to report 2 cases of pleomorphic adenoma of atypical location and then to discuss the difficulties linked to its diagnostic and therapeutic management in the Malian context. They were a 40-year-old man and a 72-year-old woman. They were admitted to the ENT department of the “Luxembourg Mere-infant” hospital for oropharyngeal swelling for the first and swelling of the palate for the second. The clinical expression was a swelling in both cases, of a hard, mobile consistency with healthy mucosa on their surface. The remainder of the physical examination was unremarkable. The diagnosis of a tumor of the oropharynx and palate was made following clinical radiological examinations. The histopathological examination of the surgical specimen made it possible to make the diagnosis of pleomorphic adenoma of the palate and the left palatine tonsil. The follow-up surgery was straightforward, with a favorable outcome. Pleomorphic adenomas are relatively rare benign tumors of the accessory salivary glands;their clinical expression remains swelling. Therapeutic management is surgery and the diagnosis is confirmed by histopathological examination.
文摘Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are one spectrum of rare cutaneous neoplasms that typically arise in sun-exposed skin of older population. AFX/PDS is essentially diagnosis of exclusion requiring Immunohistochemical work-up to exclude other types of tumors. Case Report: We present a case involving an ulcerated solitary lesion on the scalp of an elderly man. Histological examination revealed that the dermal tumor was composed of large pleomorphic, epithelioid, and spindle cells with clear cytoplasm. These cells were negative for cytokeratins, melanocytes and smooth muscle markers, but positive for CD10. These findings are consistent with a diagnosis of clear cell (CC) PDS. Conclusion: PDS is a low-grade malignancy that can recur locally and metastasize, which is distinguished from AFX by its larger size and the presence of aggressive histopathologic features including deeper invasion into the subcutaneous tissue, tumor necrosis, and lymphovascular and/or perineural involvement. Among several histopathologic variants, the CC variant is extremely rare with only two cases of PDS reported in the literature to date.
文摘·AIM: To detect proteomic differences in tears between adenoid cystic carcinoma(ACC) and pleomorphic adenoma(PA).·METHODS: Tear samples were collected from 4 patients with ACC, 5 with PA, and 4 control cases. Label-free analysis and parallel reaction monitoring(PRM) were used to screen and validate the tear proteome. Gene Ontology(GO) annotation and Kyoto Encyclopedia of Genes and Genomes(KEGG) were conducted for bioinformatics analysis.·RESULTS: In total, 1059 proteins in tear samples were identified by label-free analysis. Between ACC and PA, 415 differentially expressed proteins were detected. Based on the GO annotation, enzyme regulator activity and serinetype endopeptidase inhibitor activity in the molecular function category, blood microparticle and extracellular matrix in the cellular component category, and response to nutrient levels in the biological process category were most predominant. By KEGG pathway annotation, the different proteins between ACC and PA mainly par ticipated in complement and coagulation cascades, amoebiasis, African trypanosomiasis and cholesterol metabolism. Eight proteins with mostly significant differences were verified by PRM, and five proteins with more than 10-fold increases in ACC compared with PA, including integrin β, α-2-macroglobulin, epididymal secretory sperm binding protein Li 78p, RAB5C, and complement C5, were identified.·CONCLUSION: The combined tools of label-free analysis and PRM are very effective and efficient, especially for samples such as tears. Some proteomic dif ferences in tears between ACC and PA are identified and these protein candidates may be specific biomarkers for future exploration.
文摘Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,progresses rapidly,and is prone to remote metastasis.Currently,there is no standard protocol for the treatment of thyroid sarcomas,and most treatment effects are unsatisfactory.Argon-helium cryoablation is an important method of local treatment that is widely used in patients with unresectable advanced tumors.However,owing to the low incidence of thyroid sarcomas,there are no relevant literature reports on the treatment of thyroid sarcomas using cryoablation in China.This study reports the case of a patient with undifferentiated pleomorphic sarcoma of the thyroid gland who was treated with argon-helium cryoablation,and the immediate outcome was good.Based on a review of relevant literature,we discussed the effectiveness and safety of argon-helium cryoablation treatment,to provide clinical guidance and references for the treatment of patients with thyroid sarcoma.
文摘Background: Mucoepidermoid carcinoma ex-pleomorphic adenoma (MECxPA) is an extremely rare salivary gland malignancy. With only nine prior reported cases, this entity represents a challenging histopathological diagnosis. Methods: We present a case of a 71-year-old male with an enlarging left neck mass over several months. CT showed both a parapharyngeal space mass and a separate level II neck mass. Results: The patient underwent resection of the left parapharyngeal mass and ipsilateral selective level II-IV lymphadenectomy. The final pathologic diagnosis was metastatic high-grade mucoepidermoid carcinoma ex-pleomorphic adenoma. Conclusions: We describe a novel presentation of high-grade mucoepidermoid carcinoma ex-pleomorphic adenoma as a metastatic parapharyngeal mass.
文摘Giant cell tumors of the pancreas come in three varieties-osteoclastic,pleomorphic,and mixed histology.These tumors have distinctive endoscopic,clinical,and cytological features.Giant cell tumors have a controversial histogenesis,with some authors favoring an epithelial origin and others favoring a mesenchymal origin.The true origin of these lesions remains unclear at this time.These are also very rare tumors but proper identification and differentiation from more common pancreatic adenocarcinoma is important.The risk factors of these tumors and the prognosis may be different from those associated with standard pancreatic adenocarcinoma.Recognition of these differences can significantly affect patient care.These lesions have a unique appearance when imaged with endoscopic ultrasound(EUS),and these lesions can be diagnosed via EUS guided Fine Needle Aspiration(FNA).This manuscript will review the endoscopic,clinical,and pathologic features of these tumors.
文摘BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been reported in the English literature. METHODS:We report a rare case of a hepatic UPS presenting as a unilocular cystic lesion with respect to histopathologic features,the newly revised diagnostic criteria,and the differential diagnosis. RESULTS:A 60-year-old man presented for evaluation of epigastric pain of 7 months duration.Abdominal computed tomography revealed a hypodense,unilocular cystic mass in the right lobe of the liver,measuring 14.0×11.1 cm.A right lobectomy was performed.Grossly,the cut surface showed a single,well-circumscribed unilocular cystic tumor mass containing dark red-brown necrotic debris and blood clots, which occupied most of the mass.Microscopically,the tumor consisted of haphazardly arranged mononuclear pleomorphic tumor cells,admixed with abundant osteoclast- like multinucleated giant cells.Immunohistochemically,the tumor cells expressed vimentin only.The histopathologic and immunohistochemical findings were compatible with a UPS.The patient is alive and well 41 months after surgery without recurrence. CONCLUSIONS:Clinically,most of the hepatic UPSs are solid masses.Only two cases have presented as multilocular cystic masses.A primary hepatic UPS presenting as a unilocular cyst has never been reported.A UPS should be included in the differential diagnosis of unilocular cystic lesions in the liver.
文摘BACKGROUND Carcinoma ex pleomorphic adenoma (CXPA) is defined as a malignant salivary gland tumor arising from a primary or recurrent pleomorphic adenoma.Only three cases of CXPA of the trachea have been reported in the literature.CASE SUMMARY We report a case of tracheal CXPA in a 55-year-old woman,who presented with a more than 3-mo history of progressive dyspnea.Computed tomography of the neck and thorax revealed an inhomogeneous,broad-based lesion arising from the tracheal wall on the right side.Endoscopy revealed a subglottic neoplasm causing up to 90% luminal stenosis.The tumor was resected using a highfrequency electrosurgical snare combined with argon plasma coagulation.Histopathology and immunohistochemistry revealed that the tumor was a CXPA of the trachea.CONCLUSION We report the fourth case of tracheal CXPA,and present the first instance of resection of CXPA using high-frequency electrosurgical snare and laser ablation.We also discuss the pathogenesis,diagnosis,histopathology,and systemic therapy of this rare disease.
基金Supported by the National Natural Science Foundation of China (No.30371515)
文摘AIM: To investigate C-myc, Ki-67, pan-cytokeratin, and vimentin immunohistochemical features of carcinoma ex pleomorphic adenoma(Ca-ex-PA) and pleomorphic adenoma(PA) in the lacrimal gland in order to find some clues in the differential diagnosis between them.METHODS: We reviewed microscopic slides and clinical records of 64 cases of PA and 15 cases of Ca-ex-PA in the lacrimal gland. Immunohistochemical antibodies for C-myc, Ki-67, pan-cytokeratin, and vimentin were employed.RESULTS: Median age of PA was 43.2 y(from 21 to 75). The 35 patients(54.7%) were male and 29 patients(45.3%) were female. For the PAs, the average positivity of C-myc was 4.6%;the average proliferation index of Ki-67 was 3.2%;pan-cytokeratin was positive in ductal cells, and vimentin was positive in myoepithelial cells. Median age of Ca-ex-PA was 54.3 y(from 26 to 76). There were 7 male patients(46.7%) and 8 female patients(53.3%). Among 15 Ca-ex-PAs, there were 6 myoepithelial carcinomas, 4 adenocarcinomas, 3 epithelial-myoepithelial carcinomas, and 2 squamous cell carcinomas. For the Ca-ex-PAs, the average positivity of C-myc was 36.4%;the average proliferation index of Ki-67 was 29.2%;pan-cytokeratin was positive in all cases, and vimentin was positive in myoepithelial carcinomas.CONCLUSION: PA has a lower positivity of C-myc and Ki-67, while Ca-ex-PA had a higher positivity of these two biomarkers. These four biomarkers as a set could provide valuable clues in the differential diagnosis between Ca-exPA and PA. Our results indicate that the activation of C-myc could play an important role in the pathogenesis of Ca-exPA and PA.
文摘BACKGROUND Pleomorphic lipoma (PL) is a rare benign mesenchymal tumor occurring predominantly in middle-aged and elderly men. It is typically found in the subcutaneous tissue of the posterior neck, back, and shoulders. There have also been a few reported cases in the face, scalp, and upper extremities. Currently, there is no report on its occurrence in the anterior mediastinum. CASE SUMMARY Herein, we report the case of a 67-year-old woman diagnosed with PL in the anterior mediastinum. The tumor was removed by thoracoscopic surgery. There was no recurrence during the 24-mo follow-up period, and the prognosis was good. Most PL are located on the skin surface. However, they may also occur within the body, even in the mediastinum. CONCLUSION PL occurring in the anterior mediastinum is rare, and it may be differentiated from other tumors.
基金Beijing Hospitals Authority’Ascent Plan (No.DFL20190201).
文摘AIM:To monitor the prognosis of patients with lacrimal gland pleomorphic adenoma(LGPA)following surgical resection within the past 10y.The factors affecting patient prognosis are analyzed and the experience of surgical treatment is summarized.METHODS:In this retrospective,comparative case series,clinical records from 109 cases of LGPA treated chiefly at the same institution between November 2009 and May 2019 were reviewed.All 109 patients underwent surgery,histopathological examination,and imaging examination.For patients who underwent surgery for the first time,LGPA could be resected completely,including tumor and capsule tissues,using a surgical approach via the eyebrow arch or double eyelid crease.RESULTS:The ratio of males to females was 1:1.60,the ages ranged from 19 to 74 years old with a mean age of 43.64±13.07 years old,and the ratio of left to right eyes was 1:1.37.A total of 109 patients underwent surgical excision and five of these收稿日期radiotherapy after malignant transformation.Of these patients,15 were lost to follow up within the April 1,2020 deadline and 1 was diagnosed as a recurring pathology.The 5-year recurrence rate for 86 patients who underwent initial surgery was 7.27%.Single factor analysis revealed that the course of disease,bone destruction,invasion of surrounding tissues,tumor size,capsule integrity,and expression of Ki-67 were statistically significant(P<0.05).Binomial Logistic regression analysis showed that capsule integrity was a risk factor influencing recurrence(P=0.008).CONCLUSION:LGPA has a risk of recurrence and potential for malignant transformation.Complete removal of the tumor and capsule in the initial surgery is a key factor in preventing recurrence.
文摘BACKGROUND Pleomorphic rhabdomyosarcoma(RMS)of the spermatic cord is a group of rare neoplasms,and a secondary hydrocele testis occasionally occurs.The misdiagnosis of paratesticular mass may lead to a therapeutic delay.CASE SUMMARY A 79-year-old man presented to our clinic complaining of a 1-mo history of painless scrotal swelling.Physical examination revealed approximately a 15 cm×10 cm×5 cm inguinal mass with limited mobility.Contrast-enhanced magnetic resonance imaging showed a hydrocele testis,several enlarged inguinal lymph nodes,and a heterogeneously enhanced lesion with a relatively well-defined margin in the left inguinal region.Due to the imaging findings,he was diagnosed with pleomorphic RMS and received a wide resection of the mass,an inguinal incision with a high section of the left spermatic cord,and a left radical orchiectomy.He experienced local relapse 1 mo postoperatively and received radiotherapy and anlotinib hydrochloride-based immunotherapy as adjuvant therapy.The patient died 3 mo after the surgery.CONCLUSION The optimal interventions for advanced-stage pleomorphic RMS patients should be investigated by more preclinical studies and clinical trials.Physicians need to be aware of the occurrence of pleomorphic RMS in unusual locations,especially when accompanied by a hydrocele testis.
文摘We present a case of spontaneous undifferentiated/unclassified sarcoma,of a pleomorphic subtype formerly known as malignant fibrous histiocytoma(UPS/MFH),arising from the pancreas of a laboratory rat.The mass was excised after laparotomy from a 6‐month‐old female laboratory Wistar rat.It presented a giant multilobulated mass of irregular shape,which had arisen from the pancreas and occupied almost the entire peritoneal cavity.Histologically the tumor was characterized by a highly variable morphological pattern,with frequent transitions from storiform to pleomorphic areas.An extensive immunohistochemical examination revealed no specific lines of differentiation.Immunohistochemical positivity was observed only to MIB‐1(high Ki‐67 proliferation index),vimentin and CD68 antibodies.The diagnosis was compatible with UPS/MFH.To the best of our knowledge,the present case is the first report of a spontaneous primary UPS/MFH arising from the pancreas of a laboratory rat.
文摘BACKGROUND Myoepithelial carcinoma(MC)is a clinically rare malignancy,there is controversy regarding its etiology and its biological behavior is not fully elucidated.Extensive surgical resection is the main treatment method.We describe a case of pleomorphic adenoma(PA)with multiple postoperative recurrences after malignant transformation,and the history of the disease in this patient was more than 20 years.Complete resection during the first surgery of PA and long-term postoperative follow-up is necessary.CASE SUMMARY A 34-year-old male with PA and a history of 5 postoperative recurrences over 21 years,each surgically removed,presented 15 d ago with headache,nasal congestion,protrusion of the right eyeball and loss of vision in the right eye,with progressively worsening symptoms.The patient underwent surgery,and MC was confirmed by pathology examination.A small PA component was locally visible under light microscope.The patient had a recurrence of the tumor 2 mo after surgery and underwent surgical resection.CONCLUSION During the first operation for PA,care should be taken not to rupture the envelope to prevent tumor cell implantation,and when complete resection is not possible due to the anatomical site,postoperative radiotherapy is necessary to control the lesion and prevent infiltration and malignant transformation of the tumor to MC.Computed tomography and magnetic resonance imaging is important for establishing diagnosis and developing a treatment plan.
文摘We report a case of carcinoma ex pleomorphic adenoma of a sublingual gland in a 70-year-old man.Under a clinical diagnosis of benign salivary gland tumor,excision of the mass with the sublingual salivary gland in an en bloc fashion via an intraoral approach was performed.Histopathologically,there was a rupture of the fibrous capsule and diffuse cell-rich sheets composed of myoepithelial cells with round nuclei were also seen.Immunohistochemically,the cells that composed of cell rich sheets were positive to smooth muscle actin.Final diagnosis of myoepithelial carcinoma ex pleomorphic adenoma was made.