Clinico-pathologic conference: persistent tachypnea in a 5-week-old boy.

The patient was a 5-week-old male admitted with tachypnea and increased respiratory: distress. Mother noted increased cough and increased work of breathing. There was no fever, congestion, or nasal discharge. Prior to presenting to the emergency room, he was given a bronchodilator nebulizer treatment at home with no improvement. Two weeks prior to the current admission, he was hospitalized in the pediatric intensive care unit for approximately two weeks for similar complaints. Physical examination on the current admission was significant for minimal nasal flaring and mild subcostal retractions, with intermittent oxygen requirement. Infectious workup was negative.

Alveolar soft part sarcoma: clinical, histopathological, molecular, and ultrastructural aspects.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor occurring mainly in the adolescents and young adults. Multimodality treatment has not been effective, and excision remains the mainstay of treatment. Histopathologically, it varies little from case to case. It is composed of organoid aggregates of large polygonal cells with vesicular nuclei and abundant granular, eosinophilic cytoplasm, separated by delicate vascular channels. The line of differentiation of this unique tumor is yet undetermined, although recent advances have led to a better understanding of the genetic events underlying the pathogenesis of this tumor. The histopathological, ultrastructural, immunohistochemical, and genetic aspects of ASPS are discussed.

Restrictive dermopathy: report and review.

Restrictive dermopathy is a rare autosomal recessive disorder characterized by extreme tautness of the skin causing restricted intrauterine movement and a fetal akinesia deformation sequence. It is uniformly mostly neonatally fatal. The diagnostic findings are skin tautness with near absence of the dermal elastic fibers, usually with no or only minor anomalies of the internal organs. The exact pathogenetic mechanisms are still not known. Fewer than 50 cases have been reported. We report on a case of restrictive dermopathy and discuss the differential diagnoses.

Embryonal rhabdomyosarcoma arising in the ureter.

Embryonal rhabdomyosarcoma, botryoid variant, is a malignant tumor that usually arises in the urinary bladder and other mucosal sites. This is a case of a 4-year-old girl presenting with a mass initially thought to arise within the urinary bladder. Macroscopic and microscopic examination proved that this botryoid rhabdomyosarcoma had its origin within the left ureter.

Renal sinus myelolipoma coexistent with renal pelvis papillary transitional cell carcinoma: a case report.

A unique case of renal sinus myelolipoma presenting as a mass coexistent with papillary transitional cell carcinoma is reported. The patient was a 64-year-old man with a history of bladder transitional cell carcinoma. He presented with gross hematuria and a filling defect in the renal pelvis on computed tomography scan. Pathological findings revealed an irregular myxoid fatty mass, in addition to high-grade papillary transitional cell carcinoma. The differential diagnosis included myxoid liposarcoma, myxoid variant of malignant fibrous histiocytoma (myxofibrosarcoma), and angiomyolipoma. Immunoperoxidase staining confirmed the presence of hematopoietic cells, whereas diagnostic histological and immunohistochemical features of liposarcoma, myxofibrosarcoma, and angiomyolipoma were absent. Myelolipoma and papillary transitional cell carcinoma appear to be unrelated coexistent entities in this case.