ACTH-produzierendes Paragangliom der Nasennebenh�hlen

Lieberum, B.; Jaspers, C. A. J. J.; Münzenmaier, R.

doi:10.1007/s00106-002-0695-8

Cited by 17 publications

(13 citation statements)

References 20 publications

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“…EAS cases due to paraganglioma have been reported in only 10 patients (Table 1). [4][5][6][7][8][9][10][11][12][13] The tumour was located in the mediastinum in four of them, in paranasal sinus in three, and in the retroperitoneum in three patients. Only one patient had malignant tumours located throughout the thorax and abdomen.…”

Section: Discussionmentioning

confidence: 94%

Ectopic Cushing’s syndrome due to retroperitoneal ACTH-producing paragangliomas

Wang

et al. 2016

CUAJ

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Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing's syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH). He got clinical and biochemical recoveries without any recurrent evidence at the nine-month followup.

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Section: Discussionmentioning

confidence: 94%

Ectopic Cushing’s syndrome due to retroperitoneal ACTH-producing paragangliomas

Wang

et al. 2016

CUAJ

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“…The clinicopathology of sinonasal paraganglioma in comparison to other head and neck paraganglioma are not quite well understood, arising from middle turbinate, fronto-ethmoid air cells, [4] sphenoid [12] and maxillary sinus, [13] skull base [5] and pterygopalatine fissure, [10] they are rarely hormonally active with only three reported cases, [8,9] moreover they have no named related Paraganglions.…”

Section: Discussionmentioning

confidence: 99%

“…[2] The reported sinonasal paraganglioma are < 5% of all head and neck paraganglioma [3] with a predilection for middle age females, [4] they usually present with history of nasal obstruction associated with [5] or without epistaxis [6] and or very rarely as Cushing's syndrome due to ACTH secretion. [7][8][9] Computed tomography (CT) and magnetic resonant imaging (MRI) are the investigations of choice, [10] however, none has a pathognomonic sign for the disease. Their behavior is variable, ranging from malignant locally aggressive with metastases to simple benign expanding mass.…”

Section: Introductionmentioning

confidence: 99%

Paraganglioma of maxillary sinus associated with Addison’s disease mimicking a vascular tumour: A case report

Al-Kindy

2016

JST

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Paraganglioma of the nasal and paranasal sinuses are quite rare neuroendocrine tumours, usually hormonally inactive, presenting as nasal polyps with or without epistaxis. We present a rare case of a 23-year old male, with Addison's disease, who was referred from endocrinology department with a complaint of right nasal blockage associated with recurrent epistaxis. A provisional clinical diagnosis of vascular tumour was made, computed tomography (CT) and magnetic resonant imaging (MRI) suggested hemangioma/inverted papilloma. However, biopsy reported a highly vascular tissue mimicking angiofibroma, further immunostaining studies with Synaptophysin and S-100 confirmed the diagnosis of paraganglioma. This rare tumour in the paranasal sinuses may mimic vascular tumour and can only be confirmed with histopathological immunostains studies. According to our knowledge, this is first case reported to be associated with Addison's disease in the English literature.

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“…In contrast, there is little evidence of such a secretion by extra-adrenal pheochromocytomas (paragangliomas) [17]. Adrenocorticotrophic hormone (ACTH), beta-endorphine, pancreatic polypeptide, interleukine-6 and androgens are among the hormones secreted by paragangliomas [18,19,20,21,22,23,24,25]. To date, insulin secretion from a paraganglioma has been reported only by Fujino et al [6].…”

Section: Discussionmentioning

confidence: 99%

“…Their small size and retroperitoneal localization and paucity of hormonal secretion render them difficult to diagnose. Besides their ability to secrete epinephrine, norepinephrine, dopamine and chromogranin, these tumors can rarely produce other hormones and neurotransmitters [18,19,20,21,22,23,24,25]. Until now, there has been a single case report describing a female patient with hypoglycemic attacks secondary to an insulin-secreting paraganglioma [6].…”

Section: Introductionmentioning

confidence: 99%

Hypoglycemia due to Ectopic Release of Insulin from a Paraganglioma

Uysal

Temiz²,

Gül³

et al. 2007

Horm Res Paediatr

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Insulin-secreting pancreatic tumors and insulin-like growth hormone-secreting non-islet cell tumors can cause hypoglycemia. However, insulin-releasing paraganglioma or pheochromocytoma has almost never been reported. A 67-year-old female patient was admitted to our hospital because of headache, palpitation, perspiration, faintness, frequent sense of hunger and absent-mindedness. These intermittent symptoms had begun approximately a year before admission. On physical examination, she had high blood pressure of 150/90 mm Hg. Hormonal studies demonstrated increased urinary norepinephrine levels, and hyperinsulinemic hypoglycemia was confirmed while the patient was symptomatic. Abdominal MRI revealed a retroperitoneal mass measuring 4.5 cm in the pancreatic region. She was treated with an alpha-blocking agent to control blood pressure preceding the removal of the mass. Histopathological diagnosis was paraganglioma, and immunohistochemically insulin staining in the neoplastic cells was demonstrated. Her blood pressure normalized and hypoglycemia relieved after the operation. The patient did not have recurrence of hypoglycemia after a year of follow-up. Paraganglioma is a rare tumor of the neural crest, and co-secretion of insulin and catecholamines has been reported only by a single case report in the literature. The present patient is another case with this co-secretion.

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ACTH-produzierendes Paragangliom der Nasennebenh�hlen

Cited by 17 publications

References 20 publications

Ectopic Cushing’s syndrome due to retroperitoneal ACTH-producing paragangliomas

Ectopic Cushing’s syndrome due to retroperitoneal ACTH-producing paragangliomas

Paraganglioma of maxillary sinus associated with Addison’s disease mimicking a vascular tumour: A case report

Hypoglycemia due to Ectopic Release of Insulin from a Paraganglioma

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