ABSTRACT
Abstract Case report of a pediatric patient with a diagnosis of bony histiocytic sarcoma of the scapula, submitted to oncologic resection (Tikhoff-Linberg type II surgery), who returned to previous activities and was disease-free after 24 months of follow-up.
Resumo Relato de caso de paciente pediátrico com diagnóstico de sarcoma histiocítico ósseo em escápula, submetido à ressecção oncológica (cirurgia de Tikhoff-Linberg tipo II), com retorno às atividades prévias e livre de doença após 24 meses de seguimento.
Subject(s)
Humans , Male , Adolescent , Histiocytic Sarcoma/surgery , Histiocytic Sarcoma/pathology , Histiocytic Sarcoma/diagnostic imagingABSTRACT
Histiocytic sarcoma is a rare hematologic malignant neoplasia originating from histiocytic or dendritic cell clones. The lesions may be in nodal or extranodal sites, most commonly in the gastrointestinal tract. A small number of cases presents as unique cutaneous lesions. The definitive diagnosis is made by positivity for the immunohistochemical markers CD163, CD68, CD4 and lysozyme. The treatment is controversial, often with combined systemic chemotherapy. This is a case of cutaneous histiocytic sarcoma in an 82-year-old patient presenting two nodular lesions in the breast and right arm which were treated with simple excision and multidisciplinary follow-up, avoiding aggressive management and exhaustive investigations. Although most studies report aggressive evolution, the patient had good and stable clinical status during the twelve-month follow-up period.
Sarcoma histiocítico é uma rara entidade neoplásica maligna hematológica originada de clones celulares dendríticos ou histiocitários. As lesões podem ser nodais ou extranodais, mais comumente no trato gastrintestinal. Um pequeno número de casos apresenta lesões cutâneas exclusivas. O diagnóstico definitivo é feito pela positividade dos marcadores imunohistoquímicos CD163, CD68, CD4 e lisozima. O tratamento é controverso, geralmente com quimioterapia combinada sistêmica. Este é um caso de sarcoma histiocítico cutâneo em uma paciente de 82 anos apresentando duas lesões nodulares na mama e braço direitos, tratadas com exérese simples e seguimento multidisciplinar, evitando-se condutas intempestivas e investigações exaustivas. Apesar de a maioria dos estudos relatar evolução agressiva, a paciente apresenta quadro clínico bom e estável no período de doze meses de seguimento.
Subject(s)
Aged, 80 and over , Humans , Male , Histiocytic Sarcoma/diagnosis , Skin Neoplasms/diagnosis , Biomarkers/analysis , Follow-Up Studies , Histiocytic Sarcoma/surgery , Immunohistochemistry , Skin Neoplasms/surgery , Treatment OutcomeABSTRACT
We report a rare case of extranodal histiocytic sarcoma with multifocal gastrointestinal tract involvement, which has not been documented in the literature so far. A diagnosis of interdigitating dendritic cell/ histiocytic sarcoma was made on the preoperative gastric biopsy. Computed tomography scan revealed multifocal, circumferential gastrointestinal wall thickening involving the stomach and jejunal loops. Patient underwent distal gastrectomy with extended D1 dissection and proximal jejunal resection with gastrojejunostomy. Immunohistochemistry profile of both the gastric and jejunal tumors was similar to the preoperative gastric biopsy. The histiocytic origin of the tumor was confirmed by positive reaction of the tumor cells for CD 163. She received four cycles of CHOP chemotherapy, and is free of disease three years, following surgery.