Undifferentiated high-grade pleomorphic sarcoma of the common bile duct:A case report and review of literature

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.

Pleomorphic rhabdomyosarcoma of the vagina: A case report

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.

Pleomorphic Adenoma of the Salivary Glands: Two Atypical Locations

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.

Clear Cell Pleomorphic Dermal Sarcoma: A Case Report and Literature Review

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.

Preliminary proteomic analysis of human tears in lacrimal adenoid cystic carcinoma and pleomorphic adenoma

·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.

Argon-helium cryoablation treatment of undifferentiated pleomorphic sarcoma of the thyroid:A case report and literature review

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.

High-Grade Mucoepidermoid Carcinoma Ex-Pleomorphic Adenoma of the Parotid Gland: Case Report and Review of Literature

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.

Osteoclastic and pleomorphic giant cell tumors of the pancreas:A review of clinical,endoscopic,and pathologic features

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.

Undifferentiated pleomorphic sarcoma of the liver presenting as a unilocular cyst

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.

Immunohistochemical features of carcinoma ex pleomorphic adenoma and pleomorphic adenoma in the lacrimal gland

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.

Carcinoma ex pleomorphic adenoma of the trachea: A case report

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.

Pleomorphic lipoma in the anterior mediastinum: A case report

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.

Pleomorphic rhabdomyosarcoma of the spermatic cord and a secondary hydrocele testis:A case report

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.

Spontaneous pancreatic undifferentiated pleomorphic sarcoma in a laboratory rat: A case report

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.

Carcinoma ex pleomorphic adenoma of the sublingual gland:a case report

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.

Successful Treatment of Adult Pleomorphic Rhabdomyosarcoma in the Posterior Left Femur: A Case Report

Introduction: Rhabdomyosarcoma (RMS) is the most common childhood soft tissue sarcoma, but it represents only a small portion of soft tissue sarcoma in adult population. There is a treatment protocol based on Intergroup Rhabdomyosarcoma Study (IRS) that provides satisfactory results in treating RMS in children, but there is only limited evidence regarding the outcome and prognosis in extrapolating the IRS protocol to treat RMS in adults. We report a case of adult pleomorphic RMS treated with multidisciplinary approach and the results we have obtained. Case presentation: A 48-year-old woman was admitted in February 2011 due to a painful mass on her left thigh. Diagnosis of pleomorphic rhabdomyosarcoma was made by histopathology and immunohistochemistry. After multimodal treatment that includes Trans-Arterial Chemotherapy Infusion, Cryosurgery, and wide excision surgery, our patient remains disease-free as of the latest annual follow up examination on June 2017. Conclusions: The pleomorphic type of Rhabdomyosarcoma is very rare in adults and is often associated with a poor prognosis. In our case, a multidisciplinary approach with multimodal treatment provides excellent result, even after a routine follow up spanning through six years.

A Case of Pleomorphic Adenoma of the Parotid Gland with Multiple Local Recurrences through Facial to Cervical Regions

Pleomorphic adenoma is the most common benign parotid gland tumor. Although its local recurrence rate is known to be high, the recurrence extending to the cervical region is rare. Here we report a case of a young female (25 years old) with pleomorphic adenoma of the parotid gland which showed multiple recurrences through facial to cervical regions over a span of eight years. We also discuss how this benign tumor with a high recurrence rate has been treated in other cases, and how it should be treated.

Pleomorphic Adenoma with Exuberant Squamous Metaplasia and Keratin Cysts Mimicking Squamous Cell Carcinoma in Minor Salivary Gland

Salivary gland tumors, the second most common neoplasm of the mouth after squamous cell carcinoma, account for a significant proportion of tumors of the oral and perioral regions. An unusual case of adenoma presented as a solitary intraoral palatine mass in a 32-year-old woman is reported here. The tumor was interpreted as an unusual pleomorphic adenoma because of the presence of exuberant squamous metaplasia, clinically mimicking squamous cell carcinoma. Moreover, the presence of cystic structures filled with keratinized material was also salient feature. Pleomorphic adenomas may occasionally display focal squamous metaplastic changes;when extensive, it presents the potential for misinterpretation of the histology as indicative of well-differentiated squamous cell carcinoma.

Effective chemotherapy for submandibular gland carcinoma ex pleomorphic adenoma with lung metastasis after radiotherapy: A case report

BACKGROUND Only a few cases of carcinoma ex pleomorphic adenoma arising in the submandibular gland have ever been reported,all with a poor prognosis. The standard treatment for salivary gland carcinoma ex pleomorphic adenoma is surgical resection combined with postoperative radiotherapy,but the necessity of chemotherapy as well as the most appropriate treatment regimen for patients with distant metastasis after radiotherapy remains controversial.CASE SUMMARY This report presents the case of a 73-year-old woman who suffered submandibular gland carcinoma ex pleomorphic adenoma. She accepted surgery to remove the mass; she was found to have lung metastasis after radiotherapy.Her condition was controlled by chemotherapy with liposomal doxorubicin plus cisplatin.CONCLUSION The positive clinical outcome in the patient reveals that this chemotherapy regimen may be an alternative treatment for such a condition.

Intermittent facial spasms as the presenting sign of a recurrent pleomorphic adenoma

The intimate anatomical relationship of the facial nerve to the parotid parenchyma has a significant influence on the presenting signs and symptoms, diagnosis and treatment of parotid neoplasms.However, to our knowledge, hyperactivity of this nerve, presenting as facial spasm, has never been described as the presenting sign or symptom of a parotid malignancy.We report a case of carcinoma arising in a recurrent pleomorphic adenoma of the left parotid gland(i.e., carcinoma ex pleomorphic adenoma) that presented with hemifacial spasms.We outline the differential diagnosis of hemifacial spasm as well as a proposed pathophysiology.Facial paralysis, lymph node enlargement, skin involvement, and pain have all been associated with parotid malignancies.To date the development of facial spasm has not been reported with parotid malignancies.The most common etiologies for hemifacial spasm are vascular compression of the ipsilateral facial nerve at the cerebellopontine angle(termed primary or idiopathic)(62%), hereditary(2%), secondary to Bell's palsy or facial nerve injury(17%), and hemifacial spasm mimickers(psychogenic, tics, dystonia, myoclonus, myokymia, myorthythmia, and hemimasticatory spasm)(17%).Hemifacial spasm has not been reported in association with a malignant parotid tumor but must be considered in the differential diagnosis of this presenting symptom.