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
Dendritic cell neurofibroma with pseudorosettes (DCNWPR) is a recently proposed variant of neurofibroma with a distinctive microscopic appearance that is produced by a pseudorosette pattern formed by small, dark, lymp...Dendritic cell neurofibroma with pseudorosettes (DCNWPR) is a recently proposed variant of neurofibroma with a distinctive microscopic appearance that is produced by a pseudorosette pattern formed by small, dark, lymphocyte- like cells (Type I cells) arranged concentrically around larger cells, with pale- staining vesicular nuclei and copious faintly eosinophilic cytoplasm (Type II cells). Although DCNWPR appears not to be associated with neurofibromatosis (NF), 1 patient with DCNWPR has been described and suggested to have a form of NF because of multiple skin lesions, with 2 of them being DCNWPR as confirmed histologically. The aim of this study was to find out whether the neurofibromatosis type 2 (NF2) gene is mutated in DCNWPR. Seven histologically proven cases of DCNWPR, from which a substantial amount of archival paraffin- embedded material was available, were selected for this study. Three cases have been previously reported, including the intraneural lesion, and 4 cases were newly identified. There were 3 female and 4 male patients, ranging in age from 30 to 61 years (median, 48 yrs). All patients clinically presented with a small solitary lesion that was clinically diagnosed as fibroma or neurofibroma, and none of the patients had signs of NF. Follow- up was known for 6 patients (range, 1- 5 yrs; median, 2.5 yrs) and was uneventful in each case. Microscopically, all lesions fulfilled the criteria for DCNWPR. Exons 1 to 15 of the NF2 gene were amplified by PCR using primers previously published. The amplified fragments were purified and sequenced. The obtained sequences were computer analyzed and compared with the data of the GenBank database. No mutation was identified in 5 analyzed samples from which suitable DNA had been extracted. DCNWPR appears to have no mutation in exons 1- 15 of the NF2 gene. Given the relatively small number of cases studied, it seems premature to declare that amutation of the NF2 gene is not involved in DCNWPR, as the possibility cannot be excluded that mutations were present but remained undetected because they occurred in exons that were not examined.展开更多
This report emphasizes a carcinoid- like pattern, a previously unrecognized feature in cutaneous sebaceous neoplasms. We report 7 patients with sebaceous tumors in which neoplastic cells were arranged in a trabecular ...This report emphasizes a carcinoid- like pattern, a previously unrecognized feature in cutaneous sebaceous neoplasms. We report 7 patients with sebaceous tumors in which neoplastic cells were arranged in a trabecular and ribbon- like pattern or formed rosettes/pseudorosettes. The cases included 6 men and 1 woman, with their ages at the diagnosis ranging from 43 to 87 years (median age, 59). All patients presented with a solitary lesion. Locations were the scalp (n=6) and forearm (n=1). The carcinoid- like arrangement of neoplastic cells was the sole pattern in 4 cases, and in 3 cases the so- called labyrinthine/sinusoidal and/or rippled patterns were seen in addition. Sebaceous differentiation in the form of mature sebocytes varied from almost none to approximately 10% . Although the neoplasm appeared benign architecturally, the presence of cytologic atypia qualified 2 tumors as low- grade carcinomas. Four lesions represented sebaceomas, and in 1 case microscopic delineation between a carcinoma and sebaceoma was difficult. No neuroendocrine differentiation was demonstrated immunohistochemically, histochemically, and ultrastructurally. Electron microscopic examination performed in 1 case of carcinoma revealed lipid vacuoles in a minority of cells. There were no membrane- bound neuroendocrine granules. Rare cells contained peculiar large helioid inclusions. We conclude that the carcinoid- like pattern is another distinctive pattern indicative of sebaceous neoplasms. This pattern seems to be closely relatedtotherippledandlabyrinthine/sinusoidalpatterns,asexemplified by our cases, in which these arrangements sometimes occurred simultaneously.展开更多
Hamartoma is a neoplasm-like lesion composed of an abnormal mixture of tissues native to the area. We report on an unusual example of a hamartomatous lesion that showed an abnormal mixture of elements of the folliculo...Hamartoma is a neoplasm-like lesion composed of an abnormal mixture of tissues native to the area. We report on an unusual example of a hamartomatous lesion that showed an abnormal mixture of elements of the folliculosebaceous-apocrine unit and arrector pili muscle. A 13-year-old female presented with two nodules situated 2 cm apart on the lower leg that were present since birth. The patient reported some enlargement and slight pain of the otherwise asymptomatic lesions during 2 years, before they were surgically removed. The patient obviously had no skin problems afterwards; her 12-year follow-up was significant for leucopenia and nephrolithiasis. The biopsies from both lesions looked identical. Each lesion was biphasic, i.e. composed of a predominant mesenchymal component and epithelial elements. The mesenchymal part resembled a leiomyoma, or in minor areas, the constituting cells had a myofibroblastic appearance. Encased within the mesenchymal component were large infundibulocystic structures and plentiful grouped and scattered roundish solid epithelial nodules, duct-like, glandular, and tubular elements. Some of the epithelial structures were reminiscent of those seen in tubular apocrine adenoma; others resembled syringomatous elements. We interpreted this unusual lesion as a hamartoma of the folliculosebaceous -apocrine unit.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Dendritic cell neurofibroma with pseudorosettes (DCNWPR) is a recently proposed variant of neurofibroma with a distinctive microscopic appearance that is produced by a pseudorosette pattern formed by small, dark, lymphocyte- like cells (Type I cells) arranged concentrically around larger cells, with pale- staining vesicular nuclei and copious faintly eosinophilic cytoplasm (Type II cells). Although DCNWPR appears not to be associated with neurofibromatosis (NF), 1 patient with DCNWPR has been described and suggested to have a form of NF because of multiple skin lesions, with 2 of them being DCNWPR as confirmed histologically. The aim of this study was to find out whether the neurofibromatosis type 2 (NF2) gene is mutated in DCNWPR. Seven histologically proven cases of DCNWPR, from which a substantial amount of archival paraffin- embedded material was available, were selected for this study. Three cases have been previously reported, including the intraneural lesion, and 4 cases were newly identified. There were 3 female and 4 male patients, ranging in age from 30 to 61 years (median, 48 yrs). All patients clinically presented with a small solitary lesion that was clinically diagnosed as fibroma or neurofibroma, and none of the patients had signs of NF. Follow- up was known for 6 patients (range, 1- 5 yrs; median, 2.5 yrs) and was uneventful in each case. Microscopically, all lesions fulfilled the criteria for DCNWPR. Exons 1 to 15 of the NF2 gene were amplified by PCR using primers previously published. The amplified fragments were purified and sequenced. The obtained sequences were computer analyzed and compared with the data of the GenBank database. No mutation was identified in 5 analyzed samples from which suitable DNA had been extracted. DCNWPR appears to have no mutation in exons 1- 15 of the NF2 gene. Given the relatively small number of cases studied, it seems premature to declare that amutation of the NF2 gene is not involved in DCNWPR, as the possibility cannot be excluded that mutations were present but remained undetected because they occurred in exons that were not examined.
文摘This report emphasizes a carcinoid- like pattern, a previously unrecognized feature in cutaneous sebaceous neoplasms. We report 7 patients with sebaceous tumors in which neoplastic cells were arranged in a trabecular and ribbon- like pattern or formed rosettes/pseudorosettes. The cases included 6 men and 1 woman, with their ages at the diagnosis ranging from 43 to 87 years (median age, 59). All patients presented with a solitary lesion. Locations were the scalp (n=6) and forearm (n=1). The carcinoid- like arrangement of neoplastic cells was the sole pattern in 4 cases, and in 3 cases the so- called labyrinthine/sinusoidal and/or rippled patterns were seen in addition. Sebaceous differentiation in the form of mature sebocytes varied from almost none to approximately 10% . Although the neoplasm appeared benign architecturally, the presence of cytologic atypia qualified 2 tumors as low- grade carcinomas. Four lesions represented sebaceomas, and in 1 case microscopic delineation between a carcinoma and sebaceoma was difficult. No neuroendocrine differentiation was demonstrated immunohistochemically, histochemically, and ultrastructurally. Electron microscopic examination performed in 1 case of carcinoma revealed lipid vacuoles in a minority of cells. There were no membrane- bound neuroendocrine granules. Rare cells contained peculiar large helioid inclusions. We conclude that the carcinoid- like pattern is another distinctive pattern indicative of sebaceous neoplasms. This pattern seems to be closely relatedtotherippledandlabyrinthine/sinusoidalpatterns,asexemplified by our cases, in which these arrangements sometimes occurred simultaneously.
文摘Hamartoma is a neoplasm-like lesion composed of an abnormal mixture of tissues native to the area. We report on an unusual example of a hamartomatous lesion that showed an abnormal mixture of elements of the folliculosebaceous-apocrine unit and arrector pili muscle. A 13-year-old female presented with two nodules situated 2 cm apart on the lower leg that were present since birth. The patient reported some enlargement and slight pain of the otherwise asymptomatic lesions during 2 years, before they were surgically removed. The patient obviously had no skin problems afterwards; her 12-year follow-up was significant for leucopenia and nephrolithiasis. The biopsies from both lesions looked identical. Each lesion was biphasic, i.e. composed of a predominant mesenchymal component and epithelial elements. The mesenchymal part resembled a leiomyoma, or in minor areas, the constituting cells had a myofibroblastic appearance. Encased within the mesenchymal component were large infundibulocystic structures and plentiful grouped and scattered roundish solid epithelial nodules, duct-like, glandular, and tubular elements. Some of the epithelial structures were reminiscent of those seen in tubular apocrine adenoma; others resembled syringomatous elements. We interpreted this unusual lesion as a hamartoma of the folliculosebaceous -apocrine unit.
