Asunto(s)
Adenocarcinoma , Enfermedades Pancreáticas , Neoplasias Pancreáticas , Sarcoidosis , Humanos , Neoplasias Pancreáticas/complicaciones , Neoplasias Pancreáticas/diagnóstico , Adenocarcinoma/diagnóstico , Sarcoidosis/complicaciones , Sarcoidosis/diagnóstico , Sarcoidosis/patología , Neoplasias PancreáticasAsunto(s)
Anestesia Epidural , Anestesia Obstétrica , Escabiosis , Femenino , Embarazo , Humanos , Escabiosis/complicaciones , Mujeres EmbarazadasRESUMEN
COL1A1-PDGFB gene fusion uterine sarcoma is a recently described entity which shows some overlapping features with dermatofibrosarcoma protuberans. To date, only 4 cases have been reported in the literature. Due to its rarity, succinct clinicopathologic characteristics are yet to be established. We report a fifth case initially mistaken as a uterine fibroid which histologically proved to be a CD34 + high-grade spindle cell proliferation which on fluorescence in situ hybridization analysis displayed COL1A1-PDGFB gene rearrangement. With this case description we hope to raise awareness and aid in the characterization of this emerging entity.
Asunto(s)
Dermatofibrosarcoma , Neoplasias Cutáneas , Neoplasias de los Tejidos Blandos , Humanos , Dermatofibrosarcoma/genética , Hibridación Fluorescente in Situ , Proteínas de Fusión Oncogénica/genética , Proteínas Proto-Oncogénicas c-sis/genética , Neoplasias Cutáneas/patologíaRESUMEN
ABSTRACT: Giant cell tumor of soft tissue (GCTST) is a rare neoplasm genetically unrelated but histopathologically indistinguishable to its osseous counterpart. Histologically, GCTST is characterized as a multinodular proliferation of bland histiocytoid mononuclear cells intermixed with osteoclast-like giant cells. GCTST most commonly presents as a soft-tissue mass located in the extremities of middle-aged adults. In this report, we describe a case of a dermal GCTST arising in the periocular region of a 3-year-old girl. This is the youngest patient diagnosed with GCTST reported in the literature and is also singular because of its anatomic location: Only a handful of head and neck GCTSTs have been reported to date. Furthermore, GCTST most often presents as a superficial or deep soft-tissue mass and much less commonly as a dermal-based skin tumor, as was our case. On microscopic examination, the resected lesion demonstrated classical features including numerous osteoclast-like giant cells embedded in a background of mononuclear ovoid cells which displayed brisk mitotic activity and were surrounded by variable stromal hemorrhage. Tumor cells presented a vaguely fascicular arrangement. Immunohistochemical profile demonstrated positivity for smooth muscle actin and CD68 and negativity for desmin, myo-D1, S100, HMB-45, Melan-A, p16, and NKIC3. The unusual characteristics of this case emphasize the clinicopathologic heterogeneity of GCTST.