We report on a case of a sarcoma arising in the hyaline- vascular variant of Castleman disease (HVCD) of the skin and subcutis. The patient was a 38- year- old man who clinically presented with a subcutaneous non- fix...We report on a case of a sarcoma arising in the hyaline- vascular variant of Castleman disease (HVCD) of the skin and subcutis. The patient was a 38- year- old man who clinically presented with a subcutaneous non- fixed cyst- like mass on his right shoulder with an unremarkable prior medical history. Histologic sections showed a biphasic tumor with numerous atretic lymphoid follicles located in the deep dermis and subcutis and a spindle- cell neoplasm mainly situated in the deep subcutis and adjacent soft tissue. The atretic lymphoid component fulfilled the criteria for HVCD, whereas the spindle- cell lesion showed all the criteria for sarcoma including nuclear atypia and frequent mitotic figures. The sarcomatous componentwas diffusely positive for fascin, nerve growth factor receptor, and CD34 with focal weak reactivity for CD21 and CNA.42. Stains for CD23, CD31, CD35, CD99, ALK- 1, SMA, ASMA, desmin, factor XIIIa,AE1- AE3, EMA, bcl- 2, S- 100, Melan- A,HMB- 45, Cam 5.2, and factor VIII were negative in the neoplastic spindle cells. No monoclonal population of lymphocyteswas detected andwe could not identify EBV or HHV- 8 virus by PCR. Electron microscopy of the sarcomatous component showed spindle cells with labyrinth- like invaginations of the nucleus and numerous long, slender, interwoven cytoplasmic processes. The sarcomatous component in this case is most consistent with a poorly differentiated follicular dendritic cell sarcoma based upon the morphologic and ultrastructural findings.展开更多
This study characterizes the clinicopathological spectrum of lymphoproliferations involving the breast nipple and/or areola. Morphologic, immunohistochemical, molecular-ge-netic, and clinical features of 58 specimens ...This study characterizes the clinicopathological spectrum of lymphoproliferations involving the breast nipple and/or areola. Morphologic, immunohistochemical, molecular-ge-netic, and clinical features of 58 specimens from 56 patients were analyzed. They were re-diagnosed as cutaneous lymphoid hyperplasia (CLH, n = 44); other benign lymphoid infiltrates (OBLI, n = 8); peripheral T-cell lymphoma,not otherwise specified (n = 1); cases with overlapping features of CLH and B-cell lymphoma (n = 3), one of them composed of spindle cells. Cutaneous lymphoid hyperplasia infiltrates were dense, composed mainly of B cells forming follicles with germinal centers (GC). Cutaneous lymphoid hyperplasia frequently showed features suggesting a malignancy as coalescing follicles with non-polarized germinal centers lacking mantle zones, and smudged infiltrates of lymphoid cells spreading into collagen (often as “ Indian files” ), smooth muscle, vessel walls, and nerve sheaths. Only two cutaneous lymphoid hyperplasias recurred; otherwise all patients are without disease (mean follow-up 62 months) . Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in five, and of T-cell receptor γ gene in two cutaneous lymphoid hyperplasias using polymerase chain reaction (PCR), but the patients fared well too. In 47% of cases Borrelia burgdorferi was detected by polymerase chain reaction and/or serology, of which one was monoclonal. We conclude that cutaneous lymphoid hyperplasia is the most common lymphoproliferation of the breast nipple, rarely recognized clinically, and often overdiagnosed histologically as lymphoma.展开更多
文摘We report on a case of a sarcoma arising in the hyaline- vascular variant of Castleman disease (HVCD) of the skin and subcutis. The patient was a 38- year- old man who clinically presented with a subcutaneous non- fixed cyst- like mass on his right shoulder with an unremarkable prior medical history. Histologic sections showed a biphasic tumor with numerous atretic lymphoid follicles located in the deep dermis and subcutis and a spindle- cell neoplasm mainly situated in the deep subcutis and adjacent soft tissue. The atretic lymphoid component fulfilled the criteria for HVCD, whereas the spindle- cell lesion showed all the criteria for sarcoma including nuclear atypia and frequent mitotic figures. The sarcomatous componentwas diffusely positive for fascin, nerve growth factor receptor, and CD34 with focal weak reactivity for CD21 and CNA.42. Stains for CD23, CD31, CD35, CD99, ALK- 1, SMA, ASMA, desmin, factor XIIIa,AE1- AE3, EMA, bcl- 2, S- 100, Melan- A,HMB- 45, Cam 5.2, and factor VIII were negative in the neoplastic spindle cells. No monoclonal population of lymphocyteswas detected andwe could not identify EBV or HHV- 8 virus by PCR. Electron microscopy of the sarcomatous component showed spindle cells with labyrinth- like invaginations of the nucleus and numerous long, slender, interwoven cytoplasmic processes. The sarcomatous component in this case is most consistent with a poorly differentiated follicular dendritic cell sarcoma based upon the morphologic and ultrastructural findings.
文摘This study characterizes the clinicopathological spectrum of lymphoproliferations involving the breast nipple and/or areola. Morphologic, immunohistochemical, molecular-ge-netic, and clinical features of 58 specimens from 56 patients were analyzed. They were re-diagnosed as cutaneous lymphoid hyperplasia (CLH, n = 44); other benign lymphoid infiltrates (OBLI, n = 8); peripheral T-cell lymphoma,not otherwise specified (n = 1); cases with overlapping features of CLH and B-cell lymphoma (n = 3), one of them composed of spindle cells. Cutaneous lymphoid hyperplasia infiltrates were dense, composed mainly of B cells forming follicles with germinal centers (GC). Cutaneous lymphoid hyperplasia frequently showed features suggesting a malignancy as coalescing follicles with non-polarized germinal centers lacking mantle zones, and smudged infiltrates of lymphoid cells spreading into collagen (often as “ Indian files” ), smooth muscle, vessel walls, and nerve sheaths. Only two cutaneous lymphoid hyperplasias recurred; otherwise all patients are without disease (mean follow-up 62 months) . Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in five, and of T-cell receptor γ gene in two cutaneous lymphoid hyperplasias using polymerase chain reaction (PCR), but the patients fared well too. In 47% of cases Borrelia burgdorferi was detected by polymerase chain reaction and/or serology, of which one was monoclonal. We conclude that cutaneous lymphoid hyperplasia is the most common lymphoproliferation of the breast nipple, rarely recognized clinically, and often overdiagnosed histologically as lymphoma.
