Background: Nevoid basal cell carcinoma syndrome (NBCC) is an autosomal dominant disorder characterized by developmental abnormalities and neoplasms including basal cell carcinoma (BCC) and sarcomas (i.e. leiomyosarco...Background: Nevoid basal cell carcinoma syndrome (NBCC) is an autosomal dominant disorder characterized by developmental abnormalities and neoplasms including basal cell carcinoma (BCC) and sarcomas (i.e. leiomyosarcoma, rhabdomyosarcoma, and fibrosarcoma). Primary cutaneous carcinosarcoma(PCC), a rare tumor composed of malignant epithelial and mesenchymal components, has never been previously described in association with this syndrome. Case report: A 61- year- old Hispanic man with a history of NBCC presented with a 4 cm nodule on the right proximal medial thigh. Pathologic findings: Areas of typical BCC merged with intersecting fascicles of large atypical spindle cells that stained for vimentin and were negative for actin, desmin, CD- 34, and S- 100 protein. Scattered bizarre solitary cytokeratin- positive epithelioid cells were embedded within the fibrocytic proliferation. Conclusions: Several carcinosarcomas have been reported to contain BCC as the malignant epithelial component, but to our knowledge, this is the first report of PCC associated with NBCC. Mutation in patched tumor suppressor gene on chromosome 9q occurs in BCCs of NBCC, and aberrancies on chromosome 9q are also reported in some carcinosarcomas. It is possible that the known genetic defect on chromosome 9 in this patient contributed to the development of carcinosarcoma.展开更多
Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is an angioproliferative lesion, typically consisting of single or multiple red papules or nodules in the head and neck region. The etiology of ALHE, whet...Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is an angioproliferative lesion, typically consisting of single or multiple red papules or nodules in the head and neck region. The etiology of ALHE, whether reactive or neoplastic, is unclear. It has been well documented in the literature that human herpesvirus-8 (HHV-8) DNA is present in the majority of cases of Kaposi’s sarcoma; however, there is contradictory data regarding the association of this virus with ALHE. Methods: We performed immunohistochemical studies for HHV-8 on paraffin-embedded tissue from 23 cases of histologically confirmed ALHE. Polymerase chain reaction (PCR) analysis for HHV-8 DNA was performed on 14 of the 23 cases that had adequate remaining tissue for the procedure. The results of the immunohistochemical studies and PCR analysis were compared. Results: HHV-8 immunohistochemical studies were negative in all 23 cases of ALHE. PCR-based analysis on 14 cases failed to identify HHV-8 DNA. Conclusions: Combined data from several, small published studies are equivocal for an association between HHV-8 and ALHE. The results of our large study show no association between HHV-8 and ALHE.展开更多
An 86-year-old African-American man presented with tonicclonic seizures.Intravenous phenytoin was urgently administered into the dorsum of the right hand.The patient developed a raised purple area of discoloration aro...An 86-year-old African-American man presented with tonicclonic seizures.Intravenous phenytoin was urgently administered into the dorsum of the right hand.The patient developed a raised purple area of discoloration around the intravenous insertion site within 2 h and edema and vesiculobullous lesions of the distal forearm, hands, and fingers within 8 h.Microscopic sections from a biopsy at 12 h revealed epidermal necrosis, superficial ulceration, and a mild superficial and deep perivascular lymphoid infiltrate, associated with numerous thrombi of small vessels throughout the dermis.The findings were judged to be consistent with soft-tissue injury associated with intravenous administration of phenytoin, also termed purple glove syndrome.Purple glove syndrome, named for its distinctive purple discoloration and swelling of the hands in the distribution of a glove, is an uncommon complication of intravenous phenytoin administration through small dorsal veins of the hands.It is comprised by pain, discoloration, and edema in the vicinity of intravenous infusion of phenytoin through dorsal veins of the hand.The histopathologic features of fully developed lesions have been reported; however, early-stage findings have not been previously described, and the histogenesis of this lesion is controversial.The presence of thrombi in this earlystage lesion suggests that thrombosis plays a role in the initial pathogenesis of this condition.展开更多
文摘Background: Nevoid basal cell carcinoma syndrome (NBCC) is an autosomal dominant disorder characterized by developmental abnormalities and neoplasms including basal cell carcinoma (BCC) and sarcomas (i.e. leiomyosarcoma, rhabdomyosarcoma, and fibrosarcoma). Primary cutaneous carcinosarcoma(PCC), a rare tumor composed of malignant epithelial and mesenchymal components, has never been previously described in association with this syndrome. Case report: A 61- year- old Hispanic man with a history of NBCC presented with a 4 cm nodule on the right proximal medial thigh. Pathologic findings: Areas of typical BCC merged with intersecting fascicles of large atypical spindle cells that stained for vimentin and were negative for actin, desmin, CD- 34, and S- 100 protein. Scattered bizarre solitary cytokeratin- positive epithelioid cells were embedded within the fibrocytic proliferation. Conclusions: Several carcinosarcomas have been reported to contain BCC as the malignant epithelial component, but to our knowledge, this is the first report of PCC associated with NBCC. Mutation in patched tumor suppressor gene on chromosome 9q occurs in BCCs of NBCC, and aberrancies on chromosome 9q are also reported in some carcinosarcomas. It is possible that the known genetic defect on chromosome 9 in this patient contributed to the development of carcinosarcoma.
文摘Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is an angioproliferative lesion, typically consisting of single or multiple red papules or nodules in the head and neck region. The etiology of ALHE, whether reactive or neoplastic, is unclear. It has been well documented in the literature that human herpesvirus-8 (HHV-8) DNA is present in the majority of cases of Kaposi’s sarcoma; however, there is contradictory data regarding the association of this virus with ALHE. Methods: We performed immunohistochemical studies for HHV-8 on paraffin-embedded tissue from 23 cases of histologically confirmed ALHE. Polymerase chain reaction (PCR) analysis for HHV-8 DNA was performed on 14 of the 23 cases that had adequate remaining tissue for the procedure. The results of the immunohistochemical studies and PCR analysis were compared. Results: HHV-8 immunohistochemical studies were negative in all 23 cases of ALHE. PCR-based analysis on 14 cases failed to identify HHV-8 DNA. Conclusions: Combined data from several, small published studies are equivocal for an association between HHV-8 and ALHE. The results of our large study show no association between HHV-8 and ALHE.
文摘An 86-year-old African-American man presented with tonicclonic seizures.Intravenous phenytoin was urgently administered into the dorsum of the right hand.The patient developed a raised purple area of discoloration around the intravenous insertion site within 2 h and edema and vesiculobullous lesions of the distal forearm, hands, and fingers within 8 h.Microscopic sections from a biopsy at 12 h revealed epidermal necrosis, superficial ulceration, and a mild superficial and deep perivascular lymphoid infiltrate, associated with numerous thrombi of small vessels throughout the dermis.The findings were judged to be consistent with soft-tissue injury associated with intravenous administration of phenytoin, also termed purple glove syndrome.Purple glove syndrome, named for its distinctive purple discoloration and swelling of the hands in the distribution of a glove, is an uncommon complication of intravenous phenytoin administration through small dorsal veins of the hands.It is comprised by pain, discoloration, and edema in the vicinity of intravenous infusion of phenytoin through dorsal veins of the hand.The histopathologic features of fully developed lesions have been reported; however, early-stage findings have not been previously described, and the histogenesis of this lesion is controversial.The presence of thrombi in this earlystage lesion suggests that thrombosis plays a role in the initial pathogenesis of this condition.
