Multiple endocrine neoplasia type 2

Wohllk, Nelson; Schweizer, Heiko; Erlic, Zoran; Schmid, Kurt Werner; Walz, Martin K.; Raue, Friedhelm; Neumann, Hartmut P. H.

doi:10.1016/j.beem.2010.02.001

Cited by 98 publications

(109 citation statements)

References 86 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…However, she did not have any features of catecholamine excess or chronic hypertension that was persistent or associated with the gastrointestinal symptoms in the previous reports. It has been reported that familial pheochromocytoma could be found in multiple endocrine neoplasia (MEN) IIa (Sipple syndrome) which causes thyroid medullary carcinoma and concomitant parathyroid adenoma or hyperplasia [24], or MEN IIb (Wermer syndrome) which causes mucosal neuroma of the eyelid, lip and tongue and sometimes showing the marfanoid physique [25]. However, involvement of hereditary factors was unclear in our case because no gene analysis was performed.…”

Section: Discussionmentioning

confidence: 81%

Bilateral Pheochromocytoma Presenting with Only Gastrointestinal Complaints: A Case Report

Norouzi¹,

Fazel²,

Tavassoli³

et al. 2017

jcbr

View full text Add to dashboard Cite

Introduction: Pheochromocytoma is a rare catecholamine-secreting tumor with gastrointestinal manifestations. Case description: Herein, we report a 50 years old female patient with abdominal pain, altered bowel habit, abdominal mass lesion and laboratory features of pheochromocytoma. The patient was admitted with abdominal pain, back pain, arthralgia, weakness, lethargy, cold sweat and weight loss. She had history of diabetes mellitus, ischemic heart disease and hyperlipidemia. On admission, she was normotensive and had normal physical examination. Upper and lower endoscopies were normal. Transabdominal sonography and CT scan showed large heterogeneous masses with central necrosis and calcification in adrenal regions. In addition, 24-hour urine evaluation showed elevated vanillylmandelic acid, metanephrine and normetanephrine levels. She underwent bilateral adrenalectomy. Pathologic evaluation showed typical characteristics of pheochromocytoma. Conclusions: In any patient with abdominal pain and abdominal mass, low threshold for diagnosis of rare but often lethal pheochromocytoma is suggested.

show abstract

Section: Discussionmentioning

confidence: 81%

Bilateral Pheochromocytoma Presenting with Only Gastrointestinal Complaints: A Case Report

Norouzi¹,

Fazel²,

Tavassoli³

et al. 2017

jcbr

View full text Add to dashboard Cite

show abstract

“…6 Partial adrenalectomy, so-called 'cortical sparing adrenalectomy', has been proposed for the treatment of bilateral adrenal pheochromocytoma and to maintain adrenocortical function. 3 Lee et al carried out partial adrenalectomy using an open transabdominal approach in 14 patients with MEN2 and VHL, and 13 patients (93%) had normal postoperative cortisol levels and were weaned off replacement therapy. Recurrent pheochromocytomas developed in three patients (21%), and nine other patients were alive without recurrent tumor at a mean 90 months of follow-up.…”

Section: Discussionmentioning

confidence: 99%

“…Partial adrenalectomy, so-called 'cortical sparing adrenalectomy', has been proposed for the treatment of bilateral adrenal pheochromocytoma and to maintain adrenocortical function. 3 However, the long-term outcomes of partial adrenalectomy in patients with FP are not evident so far.…”

Section: Introductionmentioning

confidence: 99%

Postoperative Outcomes of Familial Adrenal Pheochromocytoma1

Kagawa

Hibi²,

Uchida

et al. 2011

World Journal of Endocrine Surgery

View full text Add to dashboard Cite

There are no current guidelines for the management of familial pheochromocytoma (FP). We tried to determine the optimal management of patients with FP. Among 191 patients with pheochromocytoma who underwent surgical resection between 1979 and 2010, there were 18 FP (13 different kindreds; 11 females/7 males; mean age at initial operation: 38.7 years). The 18 FP cases comprised 10 with MEN2A, 2 with MEN2B, 4 with von Hippel-Lindau disease, and 2 with FP only, and all pheochromocytomas were of adrenal origin. The number of probands and family members was 9 and 9 respectively. Mean tumor size was 6.4 cm in diameter. Simultaneous bilateral adrenalectomy was performed in 6 patients, and unilateral adrenalectomy was performed as the initial surgery in 12 patients. A metachronous contralateral adrenalectomy was performed in 3 patients, 90, 236 and 312 months after the primary operation, respectively. None of the patients received partial adrenalectomy. Among another 9 patients with unilateral adrenalectomy, contralateral pheochromocytomas were suspected in 4 cases at the initial operation. However, none of these contralateral lesions developed severe symptoms or tumor enlargement during a median follow-up of 116 months. In the remaining 5 patients, pheochromocytoma did not develop in the contralateral adrenals over a median follow-up of 80.5 months. Bilateral lesions of adrenal pheochromocytoma in familial cases occurred in 78% of cases (14/18); 9 patients (including 4 with contralateral pheochromocytoma) did not require a contralateral adrenalectomy during a median follow-up of 119 months. No patients have suffered from Addisonian crisis. The ipsilateral adrenalectomy and follow-up of contralateral small pheochromocytoma is one of the management options to preserve adrenocortical function in FP patients.

show abstract

“…Genetic testing has allowed for early detection of MEN type 2 and prophylactic thyroidectomy 55) . Tyrosine kinase inhibitors, histone deacetylase inhibitors 56) and nijmegen breakage syndromes 57) are being studied as potential new therapies for anaplastic carcinoma.…”

Section: Where We Arementioning

confidence: 99%

Thyroid Cancer: The Evolution of Treatment Options

Noguchi¹

2012

Updates in the Understanding and Management of Thyroid Cancer

View full text Add to dashboard Cite

Multiple endocrine neoplasia type 2

Cited by 98 publications

References 86 publications

Bilateral Pheochromocytoma Presenting with Only Gastrointestinal Complaints: A Case Report

Bilateral Pheochromocytoma Presenting with Only Gastrointestinal Complaints: A Case Report

Postoperative Outcomes of Familial Adrenal Pheochromocytoma1

Thyroid Cancer: The Evolution of Treatment Options

Contact Info

Product

Resources

About