RESUMO
OBJECTIVE: Pheochromocytomas are complex tumors that require a comprehensive and systematic management plan orchestrated by a multidisciplinary team. METHODS: To achieve these ends, The Mount Sinai Adrenal Center hosted an interdisciplinary retreat where experts in adrenal disorders assembled with the aim of developing a clinical pathway for the management of pheochromocytomas. RESULTS: The result was a consensus for the diagnosis, perioperative management, and postoperative management of pheochromocytomas, with specific recommendations from our team of adrenal experts, as well as a review of the current literature. CONCLUSION: Our clinical pathway can be applied by other institutions directly or may serve as a guide for institution-specific management.
Assuntos
Neoplasias das Glândulas Suprarrenais/terapia , Procedimentos Clínicos , Feocromocitoma/terapia , Neoplasias das Glândulas Suprarrenais/diagnóstico , Humanos , Feocromocitoma/diagnósticoRESUMO
A 48-year-old man of Haitian descent presented with progressive constipation, hematochezia, and weight loss. Colonoscopy and computed tomography scan revealed an obstructing colonic mass, causing intussusception and pneumatosis of the descending/upper sigmoid colon and necessitating an emergency left hemicolectomy. Gross examination revealed a 4.9-cm obstructing mass in the sigmoid colon extending through the muscularis propria. Histologically, the lesional cells were bland, spindled, with tapered and often wavy nuclei set in a loose fibromyxoid stroma. Focally, the lesional cells displayed whorling or storiform growth pattern mixed with spindle wavy cells. In many areas, the cells had bipolar cytoplasmic processes. Immunohistochemistry revealed patchy positivity for epithelial membrane antigen, CD34, vimentin, diffuse positivity for S-100, and negativity for CD117, cytokeratin (AE1/AE3), ALK1, desmin, smooth muscle actin, neuronal nuclei antigen (NeuN), and neurofilament protein. The morphology and immunohistochemical findings were consistent with hybrid perineurioma-schwannoma. Electron microscopic examination revealed the characteristic features of perineuriomal and schwannomatous differentiation. Based upon the histopathology, immunophenotype, and ultrastructure, this tumor was classified as a benign hybrid perineurioma-schwannoma, a counterpart to the tumor described in the soft tissue. This is the first case report of hybrid perineurioma-schwannoma in the colon.
Assuntos
Neoplasias do Colo/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias de Bainha Neural/patologia , Neurilemoma/patologia , Anastomose Cirúrgica , Biomarcadores Tumorais/análise , Colo/cirurgia , Neoplasias do Colo/química , Neoplasias do Colo/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/química , Neoplasias Primárias Múltiplas/cirurgia , Neoplasias de Bainha Neural/química , Neoplasias de Bainha Neural/cirurgia , Neurilemoma/química , Neurilemoma/cirurgia , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
Adrenocortical oncocytoma is exceptionally rare. Most of these tumors are benign and nonfunctioning. We report a case of functioning adrenocortical oncocytoma located in the right adrenal gland in a 53-year-old woman who presented with Cushing's syndrome. The tumor was small, with exclusively oncocytic histologic features. A discussion of this case and a review of the literature on this entity is presented.