Feocromocitoma

Pereira, Maria Adelaide Albergaria; Souza, Bruno Ferraz de; Freire, Daniel Soares; Lucon, Antônio Marmo

doi:10.1590/s0004-27302004000500022

Cited by 11 publications

(9 citation statements)

References 120 publications

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“…Different presentations are also observed in pheochromocytomas; only 5% of patients with sporadic tumors are asymptomatic, while half of the patients with MEN2a-associated pheochromocytomas will not have any symptom of the disease (11). Around one third of pheochromocytoma/paraganglioma patients carry a germline mutation (most familial cases, and 10%-20% of the sporadic cases).…”

Section: Discussionmentioning

confidence: 99%

“…Pheochromocytomas and paragangliomas are rare neuroendocrine tumors of the adrenal glands and sympathetic and parasympathetic ganglia that occur sporadically or as a part of different hereditary tumor syndromes, including MEN type 2 (MEN2), von Hippel-Lindau disease, neurofibromatosis type 1 (NF1), familial paraganglioma syndrome, and isolated pheochromocytoma/paraganglioma syndromes due to RET, VHL, NF1, SDHA, SDHB, SDHC, SDHD, SDHAF2, MAX, and TMEM127 mutations (11)(12)(13). In MEN2a, medullary thyroid cancer (MTC) occurs in nearly 100% of patients, hyperparathyroidism occurs in 20%, while pheochromocytoma is identified in 30%-50%, and it is the primary complaint in only 15% of cases.…”

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confidence: 99%

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Genetic studies in a coexistence of acromegaly, pheochromocytoma, gastrointestinal stromal tumor (GIST) and thyroid follicular adenoma

Boguszewski

Fighera

Bornschein

et al. 2012

Arq Bras Endocrinol Metab

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SUMMARYWe report on an adult woman with rare coexistence of acromegaly, pheochromocytoma (PHEO), gastrointestinal stromal tumor (GIST), intestinal polyposis, and thyroid follicular adenoma. At the age of 56, she was diagnosed with acromegaly caused by a pituitary macroadenoma, treated by transsphenoidal surgery, radiotherapy, and octreotide. During routine colonoscopy, multiple polyps were identified as tubular adenomas with high-grade dysplasia on histology. Years later, an abdominal mass of 8.0 x 6.2 cm was detected by routine ultrasound. Surgical exploration revealed an adrenal mass and another tumor adhered to the lesser gastric curvature, which were removed. Pathology confirmed the diagnosis of PHEO and GIST. PHEO immunohistochemistry was negative for GHRH. During follow-up, nodular goiter was found with normal levels of calcitonin and inconclusive cytology. Near-total thyroidectomy was performed, revealing a follicular adenoma. Her family history was negative for all of these tumor types. Genetic analysis for PHEO/paraganglioma genes (SDH A-D, SDHAF2, RET, VHL, TMEM127, and MAX), and pituitary-related genes (AIP, MEN1, and p27) were negative. Though the finding of PHEO and acromegaly with multiple other tumors could be a fortuitous coexistence, we suggest that this case may represent a new variant of MEN syndrome with a de novo germline mutation in a not yet identified gene. Arq Bras Endocrinol Metab. 2012;56(8):507-12 SUMÁRIORelatamos o caso de uma mulher com rara coexistência de acromegalia, feocromocitoma (FEO), tumor do estroma gastrointestinal (GIST), polipose intestinal e adenoma folicular de tireoide. Aos 56 anos, ela foi diagnosticada com acromegalia por um macroadenoma hipofisário, tratado com cirurgia transesfenoidal, radioterapia e octreotide. Uma colonoscopia de rotina detectou múltiplos pólipos, que à histologia eram adenomas tubulares com alto grau de displasia. Anos mais tarde, uma ecografia detectou uma massa abdominal de 8.0 x 6.2 cm, que na exploração cirúrgica era uma lesão adrenal e outro tumor aderido à pequena curvatura gás-trica. A patologia confirmou os diagnósticos de FEO e GIST. A imuno-histoquímica do FEO foi negativa para GHRH. No seguimento, encontrou-se um bócio nodular com níveis normais de calcitonina e citologia inconclusiva. Após tireoidectomia total o diagnóstico histológico foi de adenoma folicular. A história familiar era negativa para todos esses tumores. As análises gené-ticas para genes de síndromes de FEO/paragangliomas (SDH A-D, SDHAF2, RET, VHL, TMEM127 e MAX) e para hipofisárias (AIP, MEN1 e p27) foram todas negativas. Embora a presença de FEO e acromegalia com múltiplos outros tumores possa ser uma coexistência fortuita, acreditamos na possibilidade de uma nova variante de NEM com uma mutação germinativa de novo em um gene ainda não identificado Arq Bras Endocrinol Metab. 2012;56(8):507-12

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Genetic studies in a coexistence of acromegaly, pheochromocytoma, gastrointestinal stromal tumor (GIST) and thyroid follicular adenoma

Boguszewski

Fighera

Bornschein

et al. 2012

Arq Bras Endocrinol Metab

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“…A cintilografia com MIBG-I 131 , além de ser informativa quanto à localização anatômica, orienta sobre a caracterização funcional do tumor (20,21). Como se trata de um tumor ≥ 8 cm, com invasão de rim esquerdo, a cintilografia também é importante para excluir a o risco de metástase, doença extra-adrenal e multifocal (22).…”

Section: Discussionunclassified

“…Feocromocitomas podem ser malignos se for detectada a presença de metástases (linfonodos, osso, pulmão, fígado) à cirurgia (22)(23)(24)(25). O caso aqui descrito não apresentava metástases, tratando-se provavelmente de tumor benigno.…”

Section: Discussionunclassified

Mínimas alterações hormonais em paciente com grande feocromocitoma

Lima

Bandeira

et al. 2006

Arq Bras Endocrinol Metab

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We present a case report that the patient had symptoms suggesting pheochromocytoma, a large tumor (> 50 g) and a single minimally altered laboratorial test, exemplifying a diagnostic pitfall. A 31 y.o. male patient had two acute abdominal events, the last one accompanied by headache, arterial hypertension, facial flushing, perspiration and cutaneous pallor. In another admission, the patient had sustained arterial hypertension and cardiac arrhythmia. From laboratory analysis, the vanililmandelic acid was slightly modified. Scintigraphy disclosed a large adrenal mass suggesting pheochromocytoma. Histopathology confirmed this hypothesis. This report points out that patients with symptoms suggesting pheochromocytoma, even when plasma catecholamines and urinary metanephrines levels are normal, may harbor large tumors with a high catecholamines turnover or that had undergone hemorrhagic necrosis.

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“…It is a rare neuroendocrine tumour among the various endocrine causes of secondary hypertension which secretes catecholamines and other neuropeptides 2. Most of the cases are sporadic in nature although 10%–25% cases may have genetic associations like multiple endocrine neoplasia type 2, type 1 neurofibromatosis and Von Hippel-Landau disease, which implies the need for genetic testing in these cases 3. Ocular manifestations of pheochromocytoma include headache and loss of vision.…”

Section: Introductionmentioning

confidence: 99%

Resolution of grade IV hypertensive retinopathy in an adult with pheochromocytoma: post-tumor resection

et al. 2020

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A 64-year-old hypertensive woman presented with complaints of a painless, progressive decrease in vision, headache, dizziness for the last month. She was a known case of diabetes mellitus on insulin therapy. On examination, best-corrected visual acuity was 6/12 in the right eye and 6/18 in the left eye. The fundus examination in both eyes revealed features of grade IV hypertensive retinopathy. Systemic examination revealed the raised blood pressure of 200/110 mm Hg. Diagnosis of pheochromocytoma was made on the basis of increased urinary norepinephrine (892.8 mg/dL) and mass in the left adrenal gland (measuring 31×28 mm) at contrast-enhanced CT. Medical management to control hypertension was done and ultimately, she underwent left laparoscopic adrenalectomy. After 10 months of surgery, the patient was asymptomatic, blood pressure was within normal limit and her vision improved to 6/6 in both the eyes. The retinal features of hypertensive retinopathy had completely disappeared.

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Feocromocitoma

Cited by 11 publications

References 120 publications

Genetic studies in a coexistence of acromegaly, pheochromocytoma, gastrointestinal stromal tumor (GIST) and thyroid follicular adenoma

Genetic studies in a coexistence of acromegaly, pheochromocytoma, gastrointestinal stromal tumor (GIST) and thyroid follicular adenoma

Mínimas alterações hormonais em paciente com grande feocromocitoma

Resolution of grade IV hypertensive retinopathy in an adult with pheochromocytoma: post-tumor resection

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