ABSTRACT
RESUMEN Los paragangliomas son tumores originados en las células neuroendocrinas que forman el sistema nervioso autónomo. Se consideran benignos aunque pueden desarrollar malignidad, por lo que su tra tamiento es quirúrgico. La presentación de paraganglioma de ubicación mesentérica es muy inusual.
ABSTRACT Paragangliomas are rare neuroendocrine tumors that arise in the autonomic nervous system. Although these tumors are considered benign, they must be removed by surgery due to their potential malig nant transformation. Mesenteric paragangliomas are extremely rare.
Subject(s)
Humans , Female , Aged , Paraganglioma, Extra-Adrenal/surgery , Mesenteric Cyst/surgery , Tomography, X-Ray Computed , Abdominal Pain/complications , Abdomen/diagnostic imagingABSTRACT
Paraganglioma is an extra-adrenal neuroendocrine neoplasia derived from neural crest precursors that arise in association with autonomic ganglia. Also called extraadrenal pheochromocytomas, these tumors are defined by their anatomical site and whether they are hormonally functional. Paragangliomas are rare neoplasms of the female genital tract and may be located in the ovaries, uterus or cervix. Those that appear in the vulvovaginal region are extremely rare. Clinical manifestations depend on unregulated catecholamine secretion and location. Diagnosis is based on morphological and immunohistochemical findings. Surgical resection is the primary treatment for this tumor as it does not respond to chemotherapy and radiotherapy. Due to its low frequency and nonspecific symptomatology, diagnosis may be difficult. We present a case of primary vulvovaginal paraganglioma.
El paraganglioma es una neoplasia neuroendocrina extraadrenal derivada de precursores de la cresta neural, que surgen en asociación con los ganglios autónomos y, en ocasiones, también son denominados feocromocitomas extraadrenales. Se definen por su sitio anatómico y si son o no hormonalmente funcionales. Los paragangliomas son neoplasias poco frecuentes del tracto genital femenino y pueden aparecer en ovarios, útero o cuello uterino. Los que aparecen en la región vulvovaginal son extremadamente raros. Las manifestaciones clínicas dependen de la secreción no regulada de catecolaminas y la localización. El diagnóstico se basa en hallazgos morfológicos e inmunohistoquímicos. La resección quirúrgica es un tratamiento primario para este tumor, ya que no responde bien a la quimio-radioterapia. Debido a su baja frecuencia y sintomatología inespecífica, puede mostrar dificultades para realizar el diagnóstico. Se presenta un caso de paraganglioma vulvovaginal primario.
ABSTRACT
Paragangliomas are relatively rare chromaffin cell tumors which may be cured through resection. Patients with paragangliomas may develop metastatic diseases. There is no consensus regarding refractory chemotherapy for treatment of metastatic disease. In this report, we presented a case of a 43-year-old woman who was admitted to the hospital with a history of episodic headaches, diaphoresis, and weakness. Elevated plasma catecholamine levels and a right paraaortic mass were observed on computed tomography. The mass was excised, and a diagnosis of paraganglioma was confirmed. After 20 months of follow-up, local recurrence and metastases were detected in the thorax, abdomen, and skeletal system. Plasma and urinary catecholamine levels were high. Chemotherapy was administered, and no improvement was observed. Therefore, following this palliative conventional chemotherapy, sorafenib was administered for three months, and, finally, positron emission tomography showed that the patient's lesions had completely regressed.
Subject(s)
Adult , Female , Humans , Abdomen , Chromaffin Cells , Consensus , Diagnosis , Drug Therapy , Follow-Up Studies , Headache , Neoplasm Metastasis , Paraganglioma , Paraganglioma, Extra-Adrenal , Pheochromocytoma , Plasma , Positron-Emission Tomography , Recurrence , ThoraxABSTRACT
A functioning paraganglioma is a rare catecholamine-producing tumor that arises from the extra-adrenal chromaffin tissue. Recently we experienced a case in which a 42 year-old male patient with a functioning extra-adrenal paraganglioma mimicked an acute coronary syndrome. A functioning extra-adrenal paraganglioma was diagnosed by means of a biochemical study and a radiological imaging study. After stabilizing his blood pressure, using alpha adrenergic blocker, we successfully removed a 6?cm sized paraganglioma from between the aorta and the IVC in the retroperitoneal space.