ABSTRACT
Biphasic pulmonary blastoma (BPB) is an extremely rare highly aggressive malignant tumor that arises from fetal lung tissue and has the classical biphasic histology of epithelial and mesenchymal components. It is usually seen in adults with a slight male predominance and smokers. Previously grouped along with well-differentiated fetal adenocarcinoma (WDFA), and pleuropulmonary blastoma (PPB), now it is considered a separate variant and grouped under sarcomatoid neoplasms. Symptoms include chest pain, cough, hemoptysis and it is asymptomatic in at least one-third of the cases. A biopsy is essential for diagnosis and surgical excision is the treatment of choice. Prognosis is poor with 5-year survival less than 20% and recurrence occurring within 12 months of surgery. An aggressive multimodality approach is required for its management and active follow up surveillance is needed to look for recurrence.
Subject(s)
Lung Neoplasms/pathology , Positron Emission Tomography Computed Tomography/methods , Pulmonary Blastoma/diagnosis , Pulmonary Blastoma/pathology , Adult , Aftercare , Biopsy , Bronchoscopy/instrumentation , Chest Pain/diagnosis , Chest Pain/etiology , Cough/diagnosis , Cough/etiology , Fluorodeoxyglucose F18/metabolism , Hemoptysis/diagnosis , Hemoptysis/etiology , Humans , Male , Neoadjuvant Therapy/methods , Neoplasm Staging/methods , Pulmonary Blastoma/drug therapyABSTRACT
Clear cell renal cell carcinoma (RCC) is the most common malignant renal tumor in adults, while chromophobe RCC (CRCC) is the third most common. Any subtypes of RCC can undergo sarcomatoid differentiation, but heterologous differentiation in sarcomatoid area is very rare in RCC. Here a 61-year male is presented with hematuria and palpable mass. Clinicoradiologically, RCC was considered and left radical nephrectomy was performed. A well-circumscribed renal mass located in the upper pole of the left kidney with variegation and extensive areas of bony hard calcification noted, was reported as CRCC with sarcomatoid differentiation containing heterologous component. CRCC subtype has good prognosis but show dismal prognosis when associated with sarcomatous differentiation. We found 11 cases of sarcomatoid CRCC containing heterologous elements reported in indexed English literature. CRCC must be differentiated from Conventional RCC and Oncocytoma. The extensive sampling of the tumor is needed. We report a very rare case of CRCC with sarcomatoid differentiation containing heterologous elements.
