Primary anaplastic lymphoma kinase-positive large B-cell lymphoma of the left bulbar conjunctiva: A case report

BACKGROUND Anaplastic lymphoma kinase(ALK)-positive large B-cell lymphoma(LBCL)is an aggressive and rare variant of diffuse LBCL.Herein,we report an uncommon case of stage IE extranodal ALK-positive LBCL initially originating in the bulbar con-junctiva.CASE SUMMARY A 63-year-old woman presented with a mass in the left bulbar conjunctiva that had persisted for six months,accompanied by swelling and pain that had per-sisted for 3 d.Eye examination revealed an 8 mm slightly elevated pink mass in the lower conjunctival sac of the left eye.Microscopically,the tumor was com-posed of large immunoblastic and plasmablastic large lymphoid cells with scattered anaplastic or multinucleated large cells.Immunophenotypically,the neoplastic cells were positive for ALK,CD10,CD138,Kappa,MUM1,BOB.1,OCT-2,CD4,CD45,EMA,CD79a,CD38,and AE1/AE3,and negative for CD20,PAX5,Lambda,BCL6,CD30 and all other T-cell antigens.The results of gene rearrangement tests showed monoclonal IGH/IGK/IGL and TCRD rearran-gements.Fluorescence in situ hybridization studies did not reveal any BCL2,BCL6 or MYC rearrangements.Furthermore,Epstein-Barr virus was not detected by in situ hybridization in the lesions.Based on the histopathological and imaging examinations,the neoplasm was classified as stage IE ALK-positive LBCL.No further treatments were administered.At the 6,15,and 21 mo postoperative follow-up visits,the patient was in good condition,without obvious discomfort.This case represents the first example of primary extranodal ALK-positive LBCL presenting as a bulbar conjunctival mass,which is extremely rare and shares morphological and immunohistochemical features with a variety of other neo-plasms that can result in misdiagnosis.CONCLUSION Awareness of the condition presented in this case report is necessary for early and accurate diagnosis and appropriate treatment.

Diagnostic and management challenges in primary cutaneous anaplastic large cell lymphoma with necrosis,inflammation,and surgical intervention:A case report

BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other dermatological conditions.This study aims to investigate these challenges by conducting a comprehensive analysis of a case presenting with PC-ALCL,emphasizing the necessity of accurate differentiation for appropriate management.CASE SUMMARY An 89-year-old female patient with diabetes and hypertension presented with arm and abdominal ulcerated mass lesions.Diagnostic procedures included skin biopsies,histopathological assessments,and immunohistochemistry,complemented by advanced imaging techniques to confirm the diagnosis.The patient’s lesions were determined as PC-ALCL,characterized by necrosis,chronic inflammation,and a distinct immunophenotypic profile,including CD30,CD3,CD4,and EBER,CD56,MUM-1,Ki 67-positive in>80%of tumor cells,CD10,but negative for anaplastic lymphoma kinase,CD5,CD20,PAX-5,Bcl-2,Bcl-6,CD8,and CD15.Recurrence was not reported at the 6-month follow-up.CONCLUSION Accurate PC-ALCL differentiation from similar conditions is crucial for effective management and requires a multidisciplinary approach.

Anaplastic thyroid cancer: Unveiling advances in diagnosis andmanagement

The review article by Pavlidis et al published in World J Clin Oncol provides a meticulous analysis of the intricacies surrounding anaplastic carcinoma of the thyroid.Thyroid carcinoma encompasses a spectrum of diseases,each charac-terized by distinct behaviors and outcomes.Diagnostic approaches encompass a diverse array of tools.Surgery remains the pivotal treatment for anaplastic thyroid carcinoma.Radiotherapy and chemotherapy offer the best overall sur-vival in aggressive disease.Combinations of immunotherapy with targeted the-rapies,such as dabrafenib-trametinib,demonstrate potential for enhanced effect-iveness and improved survival outcomes.Multifaceted approach fuelled by precision medicine and interdisciplinary collaboration is imperative in charting a course toward improved outcomes in this formidable malignancy.

An overview of the contemporary diagnosis and management approaches for anaplastic thyroid carcinoma

Thyroid carcinoma is a complex disease with several types,the most common being well-differentiated and undifferentiated.The latter,“undifferentiated carcinoma”,also known as anaplastic thyroid carcinoma(ATC),is a highly aggr-essive malignant tumor accounting for less than 0.2%of all thyroid carcinomas and carries a poor prognosis with a median survival of 5 months.BRAF gene mutations are the most common molecular factor associated with this type of thyroid carcinoma.Recent advances in targeted biological agents,immuno-therapy,stem cell therapy,nanotechnology,the dabrafenib/trametinib com-bination therapy,immune checkpoint inhibitors(ICI)and artificial intelligence offer novel treatment options.The combination therapy of dabrafenib and tra-metinib is the current standard treatment for patients with BRAF-V600E gene mutations.Besides,the dabrafenib/trametinib combination therapy,ICI,used alone or in combination with targeted therapies have raised some hopes for improving the prognosis of this deadly disease.Younger age,earlier tumor stage and radiotherapy are all prognostic factors for improved outcomes.Ultimately,therapeutic regimens should be tailored to the individual patient based on surveillance and epidemiological data,and a multidisciplinary approach is ess-ential.

Current status of anaplastic thyroid carcinoma

In this editorial we comment on the article by Pavlidis et al,published in the recent issue of the World Journal of Oncology.We focus on the recent contributions in the management of anaplastic thyroid carcinoma,highlighting the importance of surgery and radiotherapy as first line therapies in its management and the introduction of new systemic therapies beyond chemotherapy,focused on molecular alterations,an essential step in the diagnosis and included in clinical guidelines for the selection of the ideal treatment.In contrast to other neoplasms,immunotherapy,is still beginning in studies of this pathology with encouraging results.Therefore,multimodal management of the pathology together with new drugs seems to be the logical step to increase the survival of this neoplasm.

Extra-Axial Anaplastic Pleomorphic Xanthoastrocytoma Mimicking Meningioma: A Case Report with Literature Review

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.

Primary bone anaplastic lymphoma kinase positive anaplastic largecell lymphoma: A case report and review of the literature

BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare type of PBL.The aim of this report is describe the symptoms,diagnosis,and treatment of primary bone ALK-positive ALCL.CASE SUMMARY A 66-year-old man presented to our hospital with neck and shoulder pain and intermittent fever that lasted for 1 mo.After extensive evaluation,positron emission tomography-computed tomography(CT)examination showed multiple osteolytic bone lesions without other sites lesions.CT-guided biopsy of the T10 vertebral body was performed,and the pathology results showed that neoplastic cells were positive for ALK-1,CD30,and CD3.A diagnosis of primary bone ALK positive ALCL was ultimately made.The patient was in partial response after four cycle soft cyclophosphamide,doxorubicin,vincristine,and prednisone chemotherapy,and we planned to repeat the biopsy and radiological examination after completion of the fifth cycle of therapy.CONCLUSION Primary bone ALK positive ALCL is a rare disease and physicians should keep in mind that ALCL can present with isolated osseous involvement without nodal involvement,and lymphoma should be considered in the differential diagnosis of primary bone lesions.

Sarcoidosis mimicking metastases in an echinoderm microtubuleassociated protein-like 4 anaplastic lymphoma kinase positive nonsmall-lung cancer patient:A case report

BACKGROUND Rearrangements of the anaplastic lymphoma kinase(ALK)gene(ALK-positive)represent an oncogenic driver in approximately 3%-5%of non-small-lung cancer(NSCLC)patients.Sarcoidosis is a multisystem disease,and its reported incidence in Asia is 1 or less per 100000 people per year.The co-occurrence of sarcoidosis and ALK-positive NSCLC is rare,and ALK-positive lung cancer is likely to spread quickly.Therefore,the co-occurrence of sarcoidosis is more easily misdiagnosed as metastatic lung cancer by radiological examination.CASE SUMMARY A 50-year-old man had a nodule in the left superior lobe,many small nodules in left superior and right lungs,and enlarged bilateral hilar,mediastinal,and right supraclavicular lymph nodes.Computed tomography-guided pulmonary biopsy of the nodule in the left superior lobe revealed echinoderm microtubuleassociated protein-like 4 gene-ALK positive NSCLC with concomitant noncaseating granuloma.This patient was treated with crizotinib.Thirty days later,a chest computed tomography scan revealed a dramatic decrease in the size of the left superior lobe nodule;however,the lesions in the right lung progressed.The right supraclavicular lymph nodes showed granulomas,and no tumor cells were identified in the specimens. The angiotensin-converting enzyme level was high.After 1 wk of methylprednisolone treatment, a significant response of all lesionswas revealed. Following radical resection of the lung cancer, noncaseatinggranulomas were observed in both lung tissues and lymph nodes, which resultedin a diagnosis of echinoderm microtubule-associated protein-like 4-ALK positiveNSCLC accompanied with sarcoidosis.CONCLUSIONOur experience illustrates that pathological evidence is needed to confirmmetastatic disease, especially when some suspected metastatic lesions arenegative for malignancy.

Acute leukemic phase of anaplastic lymphoma kinase-anaplastic large cell lymphoma: A case report and review of the literature

BACKGROUND Anaplastic large cell lymphoma(ALCL)is a rare and heterogeneous malignant tumor,which is classi
ed as anaplastic lymphoma kinase(ALK)positive ALCL and ALK-ALCL.Many patients are diagnosed with ALCL at the stage of bone marrow involvement.However,ALCL patients with clinical manifestations consistent with acute leukemia are relatively rare.CASE SUMMARY In this report,the patient did not receive appropriate diagnosis and treatment despite a two-year history of lymph node enlargement.Hereafter,she was admitted for B symptoms and was diagnosed as ALK-ALCL by lymph node biopsy.Then,the disease progressed to leukemia without any treatment after 2 mo.The proportion of lymphoma cells in bone marrow was as high as 96%,and the proportion of peripheral blood was 84%.She also had clinical manifestations similar to acute leukemia.After completion of chemotherapy,she developed granulocytopenia and fever and died from septicemia.CONCLUSION ALCL with leukemic presentation is a late manifestation of lymphoma with low chemotherapy tolerance and poor prognosis.

Anaplastic carcinoma of the pancreas: Case report and literature review of reported cases in Japan

We report a case of a 64-year-old woman with anaplastic carcinoma of the pancreas(ACP) with cyst formation and review 60 ACP cases reported in Japan. In 20% of cases, laboratory tests revealed severe anemia(hemoglobin level < 10.0 g/d L) and elevated leucocyte counts(> 12000/mm3), which were likely attributable to rapid tumor growth, intratumoral hemorrhage, and necrosis. Elevated serum CA19-9 levels were observed in 55% of cases. Cyst-like structures were observed on imaging in 47% of cases, and this finding appears to reflect subsequent cystic degeneration in the lesion. Macroscopically, hemorrhagic necrosis was observed in 77% of cases, and cyst formation was observed in 33% of cases. ACP should be considered when diagnosing pancreatic tumors with a cyst-like appearance, especially in the presence of severe anemia, elevated leucocyte counts, or elevated serum CA19-9 levels.

Anaplastic thyroid carcinoma:A comprehensive review of current and future therapeutic options

Anaplastic thyroid carcinoma(ATC)is the rarest,but deadliest histologic type among thyroid malignancies,with a dismal median survival of 3-9 mo.Even though ATC accounts for less than 2%of all thyroid tumors,it is responsible for 14%-39%of thyroid carcinoma-related deaths.ATC clinically presents as a rapidly growing mass in the neck,associated with dyspnoea,dysphagia and vocal cord paralysis.It is usually locally advanced and often metastatic at initial presentation.For operable diseases,the combination of radical surgery with adjuvant radiotherapy or chemotherapy,using agents such as doxorubicin and cisplatin,is the best treatment strategy.Cytotoxic drugs for advanced/metastatic ATC are poorly effective.On the other hand,targeted agents might represent a viable therapeutic option.Axitinib,combretastatin A4,sorafenib and imatinib have been tested in small clinical trials of ATC,with a promising disease control rate ranging from 33%to 75%.Other clinical trials of targeted therapy for thyroid carcinoma are currently ongoing.Biological agents that are under investigation include pazopanib,gefitinib and everolimus.With the very limited therapeutic armamentarium available at the present time,targeted therapy constitutes an exciting new horizon for ATC.In future,biological agents will probably represent the standard of care for this aggressive malignancy,in the same fashion as it has recently occurred for other chemo-refractory tumors,such as kidney and hepatic cancer.

Clinical analysis of primary anaplastic carcinoma of the small intestine

Primary anaplastic carcinoma is a rare variant of small intestinal cancer. Most reports of primary anaplastic carcinoma of the small intestine are isolated case reports, therefore the clinicopathological features, therapeutic management, and surgical outcome of this tumor type remain unclear. This review analyzes the available clinical characteristics of primary anaplastic carcinoma of the small intestine and investigates key differences from differentiated adenocarcinoma of the small intestine. A Medline search was performed using the keywords ＇small intestine＇ and ＇anaplastic carcinoma＇ or ＇undifferentiated carcinoma＇. Additional articles were obtained from references within the papers identified by the Medline search. The literature revealed a poor prognosis for patients who underwent surgical resection for anaplastic carcinoma of the small intestine, which gave a 3-year overall survival rate of 10.8% and a median survival time of 5.0 mo. The literature suggests that anaplastic carcinoma~ is markedly more aggressive than differentiated adenocarcinoma of the small intestine. Surgical resection with the aim of complete tumor removal provides the only beneficial therapeutic option for patients with anaplastic carcinoma of the small intestine, because chemotherapy and radiation therapy have no significant effect on the rate of survival. However, despite complete tumor resection, most patients with anaplastic carcinoma of the small intestine are at great risk of disease recurrence. Multicenter clinical trials are expected to provide additional therapeutic strategies and establish the efficacy of multimodality adjuvant therapy. This report also highlights the importance of a systematic diagnostic approach for anaplastic carcinoma of the small intestine.

Primary pancreatic anaplastic large cell lymphoma, ALK negative: A case report

We present the fourth case of a primary pancreatic anaplastic large cell lymphoma (ALCL), ALK-. An 80-year-old man was admitted to our clinic for further investigation of a fever of unknown origin. He noted anorexia, weight loss and fatigue. His laboratory tests showed anemia and a great elevation of ESR, LDH, and β2 microglobulin. In CT and MRI scan, a soft tissue mass in the pancreas was observed. A repeated endoscopy after his admission revealed an ulcerated mass-like deformity of the duodenal bulb. Explorative laparotomy confirmed a diffuse spread of an unresectable malignant pancreatic mass extending to the adjacent organs. Duodenal and surgical biopsies identified an ALCL of T-cell lineage, ALK-. The patient died in the Intensive Care Unit due to hemodynamic instability.Our case is the first one indicating that primary pancreatic lymphoma should be suspected in a patient with pancreatic mass and elevated serum LDH and β2 microglobulin.

Clinicopathologic characteristics and therapeutic responses of Chinese patients with non-small cell lung cancer who harbor an anaplastic lymphoma kinase rearrangement

Introduction: The rearrangement of the anaplastic lymphoma kinase(ALK) gene accounts for approximately 1%–6% of lung adenocarcinoma cases and deines a molecular subgroup of tumors characterized by clinical sensitivity to ALK inhibitors such as crizotinib. This study aimed to identify the relationship between ALK rearrangement and the clinico?pathologic characteristics of non?small cell lung cancer(NSCLC) and to analyze the therapeutic responses of crizotinib and conventional chemotherapy to ALK rearrangement in NSCLC patients.Methods: A total of 487 lung cancer patients who underwent testing for ALK rearrangement in our department were included in this study. ALK rearrangement was examined by using fluorescence in situ hybridization(FISH) assay.Results: Among the 487 patients, 44(9.0%) were diagnosed with ALK rearrangement by using FISH assay. In 123 patients with adenocarcinoma who were non?smokers and of a young age(≤58 years old), the frequency of ALK rearrangement was 20.3%(25/123). Short overall survival(OS) was associated with non?adenocarcinoma tumor type(P = 0.006), poorly diferentiated tumors(P al growth factor rece= 0.001), advanced?stage tumors(P < 0.001), smoking history(P ptor(EGFR)(P = 0.008), and wild?type epidermrter time to cancer p= 0.008). Moreover, patients with poorly diferentiated and advanced?stage tumors had a shorogression compared with those with well diferentiated(P = 0.023) and early?stage tumors(P = 0.001), respectively.Conclusions: ALK?rearranged NSCLC tends to occur in younger individuals who are either non?smokers or light smokers with adenocarcinoma. Patients with ALK rearrangement might beneit from ALK inhibitor therapy.

A case report of anaplastic carcinoma of the pancreas with remarkable intraductal tumor growth into the main pancreatic duct

We herein report a case of anaplastic carcinoma of the pancreas with remarkable intraductal tumor growth into the main pancreatic duct.A 76-year-old male was referred to our hospital for treatment of a pancreatic tumor.Preoperative examinations revealed a poorly defined tumor in the main pancreatic duct in the body of the pancreas,accompanied with severe dilatation of the main pancreatic duct,which was diagnosed as an intraductal papillary-mucinous neoplasm.We performed distal pancreatectomy and splenectomy.The pathological examination revealed that the tumor consisted of a mixture of anaplastic carcinoma(giant cell type)and adenocarcinoma in the pancreas.There was a papillary projecting tumor composed of anaplastic carcinoma in the dilated main pancreatic duct.The patient is now receiving chemotherapy because liver metastasis was detected 12 mo after surgery.In this case,we could observe a remarkable intraductal tumor growth into the main pancreatic duct.We also discuss the pathogenesis and characteristics of this rare tumor with specific tumor growth.

Combined targeted therapy and immunotherapy in anaplastic thyroid carcinoma with distant metastasis: A case report

BACKGROUND Anaplastic thyroid carcinoma(ATC),also called undifferentiated thyroid cancer,is the least common but most aggressive and deadly thyroid gland malignancy of all thyroid cancers[1].It has poor prognosis,and is the leading cause of death from malignant thyroid tumors.The one-year survival rate is 20%,with a median overall survival(OS)of only 5 mo[2].The aim of this report is to provide our experience in the diagnosis and treatment of ATC.CASE SUMMARY A patient with a thyroid mass underwent surgical treatment after developing symptoms of hoarseness.The resected tumor was pathologically diagnosed as ATC.Imaging examination revealed organ and lymph node metastasis.After multiple cycles of chemotherapy and local radiotherapy,the metastases were not relieved and gradually increased in size and new metastases appeared.The patient immediately received immunotherapy combined with targeted therapy.During treatment,immune-related adverse reactions occurred,which were improved after symptomatic treatment,and tolerated by the patient.The OS of the patient was more than 30 mo after immunotherapy combined with targeted therapy.CONCLUSION For metastatic ATC,surgical treatment,radiotherapy and chemotherapy have no significant effect on remission of the disease.However,immunotherapy has made a breakthrough in the treatment of ATC。

Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma masquerading as Behcet’s disease: A case report and review of literature

BACKGROUND Anaplastic large cell lymphoma(ALCL)is a CD30-positive T cell lymphoma,a rare type of non-Hodgkin lymphoma.The current World Health Organization classification system divides ALCLs into anaplastic lymphoma kinase(ALK)-positive and ALK-negative groups.ALCL rarely presents in the gastrointestinal tract.CASE SUMMARY A 54-year-old male was admitted to the department of gastroenterology for abdominal pain.He presented with lower abdominal pain,diarrhea and recurrent oral and penile ulcers.He was misdiagnosed with Behcet's disease and treated with prednisone.But after one month,he was hospitalized in another hospital for reexamination.This time,the lesion on the penis was biopsied for histological examination.The final pathological diagnosis was ALCL,ALKnegative.The patient was treated with cyclophosphamide,doxorubicin,vincristine,prednisolone chemotherapy.However,he died within one month.CONCLUSION Gastrointestinal ALCL needs to be considered in the differential diagnosis to avoid delaying treatment.Repeated biopsy is the most important for early diagnosis and treatment.

Update on current diagnosis and management of anaplastic thyroid carcinoma

Well-differentiated thyroid carcinoma has a favorable prognosis with a 5-year survival rate of over 95%.However,the undifferentiated or anaplastic type accounting for<0.2%,usually in elderly individuals,exhibits a dismal prognosis with rapid growth and disappointing outcomes.It is the most aggressive form of thyroid carcinoma,with a median survival of 5 mo and poor quality of life(airway obstruction,dysphagia,hoarseness,persistent pain).Early diagnosis and staging are crucial.Diagnostic tools include biopsy(fine needle aspiration,core needle,open surgery),high-resolution ultrasound,computed tomography,magnetic resonance imaging,[(18)F]fluoro-D-glucose positron emission tomography/computed tomography,liquid biopsy and microRNAs.The BRAF gene(BRAF-V600E and BRAF wild type)is the most often found molecular factor.Others include the genes RET,KRAS,HRAS,and NRAS.Recent management policy is based on surgery,even debulking,chemotherapy(cisplatin or doxorubicin),radiotherapy(adjuvant or definitive),targeted biological agents and immunotherapy.The last two options constitute novel hopeful management modalities improving the overall survival in these otherwise condemned patients.Anti-programmed death-ligand 1 antibody immunotherapy,stem cell targeted therapies,nanotechnology achievements and artificial intelligence implementation provide novel promising alternatives.Genetic mutations determine molecular pathways,thus indicating novel treatment strategies such as anti-BRAF,anti-vascular endothelial growth factor-A,and anti-epidermal growth factor receptor.Treatment with the combination of the BRAF inhibitor dabrafenib and the MEK inhibitor trametinib has been approved by the Food and Drug Administration in cases with BRAF-V600E gene mutations and is currently the standard care.This neoadjuvant treatment followed by surgery ensures a twoyear overall survival of 80%.Prognostic factors for improved outcomes have been found to be younger age,earlier tumor stage and radiation therapy.A multidisciplinary approach is necessary,and the therapeutic plan should be individu alized based on surveillance and epidemiology end results.

Anaplastic carcinoma associated with a mucinous cystic neoplasm of the pancreas during pregnancy:Report of a case and a review of the literature

Oncogenesis of anaplastic carcinoma of the pancreas is a subject of controversy, because it shows sarcomatous nature with extremely poor prognosis. We herein report an unusual case of anaplastic carcinoma occurring with a recurrent mucinous cystic neoplasm in a 38-year-old female. A 10-cm retroperitoneal cystic mass was pointed out in the first pregnancy and a probable diagnosis of mucinous cystic neoplasm was made in October 2000. She refused surgery first and delivered her baby uneventfully. During her second pregnancy in 2002, however, she presented hematemesis and underwent urgent distal pancreatectomy, splenectomy and partial resection of the gastric wall where the tumor perforated. A diagnosis of borderline-type mucinous cystic neoplasm with ovarian-like stroma was made. Nine months later, CT visualized a recurrent cystic tumor near the pancreatic stump, which was subsequently resected. Pathology revealed that the tumor was composed of two different components of borderline-type mucinous cystic neoplasm and anaplastic carcinoma. The latter was intensely positive for vimentin, CD68, p53 and focally for cytokeratin, suggesting both sarcomatous and carcinomatous differentiation. She survived four years after the second surgery without tumor recurrence. Although the origin of anaplastic carcinoma has not been determined yet, it should be remembered that anaplastic carcinoma can occur in association with mucinous cystic neoplasm of more benign histology.

Rare anaplastic sarcoma of the kidney:A case report

BACKGROUND Anaplastic sarcoma of the kidney(ASK)is a rare and newly recognized renal neoplasm.The tumor usually is extensive and cystic,characterized by pleomorphic spindle cells with marked atypia and associated with multinucleated cells.To date,only 27 cases have been reported in the literature.The authors present an additional case and summarize the relevant knowledge in the literature.CASE SUMMARY A 27-year-old previously healthy woman presented with a palpable mass over the abdomen and right flank soreness for one year.After the computed tomography study,the patient underwent right radical nephrectomy obtaining a 1680-g tumor with a size of 18.4 cm×14.5 cm×11 cm.The tumor is chiefly composed of anaplastic spindle cells with marked nuclear atypia admixed with multinucleated cells.Immunohistochemical evaluation of tumor cells exhibited diffuse positivity for CD56,p53,and vimentin,and focally positive for desmin.The diagnosis of ASK was established.Unfortunately,a local tumor recurrence followed by a distant metastasis developed within months.The patient died 26 months after the initial surgery.Comparing to the previously 27 cases of ASK,the current case had a relatively worse prognosis,which might be potentially associated with older patient age,larger tumor size,and the lack of en-bloc resection of adjacent organs during the initial radical nephrectomy.CONCLUSION This case points out the featured pathological findings for diagnosing ASK and suggests more aggressive management for patients with ASK.