RESUMEN
There is a paradigm shift in the detection of phaeochromocytomas with more being identified as adrenal 'incidentalomas'. While majority of these individuals are asymptomatic, they are nevertheless at risk of subtle cardiovascular dysfunction and phaeochromocytoma crises. Therefore, early resection of phaeochromocytomas, even if subclinical, is recommended. However, the perioperative management can be challenging as the normotension can limit the initiation and titration of alpha-blockade. We present a man in his 60s with a subclinical phaeochromocytoma, discuss the evaluation of an incidentally discovered adrenal nodule, as well as the practical considerations in the perioperative management.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Feocromocitoma , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/cirugía , Humanos , Hallazgos Incidentales , Masculino , Feocromocitoma/diagnóstico , Feocromocitoma/cirugíaRESUMEN
BACKGROUND: Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade, cutaneous adnexal carcinoma with neuroendocrine differentiation. It is considered to be a precursor of invasive neuroendocrine type primary cutaneous mucinous carcinoma (PCMC). OBJECTIVE: To review clinicopathological literature summary of EMPSGC and associated neuroendocrine PCMC from all reported cases and compare its behavior vs non-neuroendocrine PCMC data reported in the literature. METHODS: A review of English literature of all EMPSGC cases with and without associated PCMC was carried out. RESULTS: EMPSGC was associated with invasive neuroendocrine type PCMC in 35.7% of cases. We found the recurrence rate of PCMC associated with EMPSGC of about 12.3%, which is much less than the 30% recurrence rate reported for the non-neuroendocrine subtype of PCMC. The non-neuroendocrine subtype of PCMC shows a 4% and 11% rate of distant and lymph node metastasis, respectively, vs EMPSGC-associated neuroendocrine type of PCMC, which is very indolent and only one recent case of locoregional metastasis out of 190 EMPSGC cases has been reported so far. LIMITATION: Limitation of our study includes data derivation from case reports and case series in the literature. CONCLUSION: The prognostic benefits for this cohort of patients may be considered in their overall management.
Asunto(s)
Adenocarcinoma Mucinoso/diagnóstico , Tumores Neuroendocrinos/patología , Neoplasias Cutáneas/patología , Neoplasias de las Glándulas Sudoríparas/fisiopatología , Adenocarcinoma Mucinoso/metabolismo , Adenocarcinoma Mucinoso/cirugía , Adenocarcinoma Mucinoso/ultraestructura , Adulto , Anciano , Anciano de 80 o más Años , Antígenos de Diferenciación/metabolismo , Diagnóstico Diferencial , Manejo de la Enfermedad , Neoplasias de los Párpados/patología , Femenino , Humanos , Metástasis Linfática/patología , Masculino , Persona de Mediana Edad , Cirugía de Mohs/métodos , Invasividad Neoplásica/patología , Recurrencia Local de Neoplasia/epidemiología , Pronóstico , Neoplasias de las Glándulas Sudoríparas/patologíaRESUMEN
Perivascular epithelioid cell (PEC) tumors are rare tumors of mesenchymal origin and can affect many anatomic regions. Although these tumors are usually benign, malignant variants exist. We document for the first time a malignant PEComa arising from the pleura of a 43-year-old Malay woman. The pathology of the tumor as well as differential diagnoses is discussed.