Ectopic ACTH Syndrome with Bilateral Pheochromocytoma in Multiple Endocrine Neoplasia Type 2A

Moon, Ji Mi; Kim, Yoonjung; Seo, Young Jin; Choi, Hee Youn; Kim, Joo Hyong; Park, Ju Ri; Lee, Yun Jeong; Kim, Hee Young; Kim, Sin Gon; Choi, Dong Seop

doi:10.3803/jkes.2009.24.4.265

Cited by 4 publications

(3 citation statements)

References 19 publications

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“…Other mechanisms for the development of ectopic Cushing syndrome from a pheochromocytoma have been reported, such as corticotropin-releasing hormone secretion [ 58–60 ] or mixed corticomedullary tumors [ 61 , 62 ]. Other cases have been reported as part of the MEN 2A syndrome with bilateral masses [ 63 , 64 ]. None of these entities was included in this review.…”

Section: Discussionmentioning

confidence: 99%

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Gabi

Milhem

Tovar

et al. 2018

Journal of the Endocrine Society

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Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome is rarely caused by a pheochromocytoma. We present a case of a 46-year-old woman who developed severe hypertension, hypokalemia, and typical Cushingoid features. Investigations revealed extremely high metanephrine, cortisol, and ACTH levels. Imaging showed a 3.8-cm left adrenal mass. Preoperative control of hypertension and hypokalemia was very challenging. The patient was cured after surgical removal of the adrenal mass. We followed this by a review of the literature using the databases Google Scholar and PubMed. A total of 58 cases have been reported to date. In summary, ACTH-producing pheochromocytoma is a rare condition that poses a clinical challenge in the perioperative period. It is important that physicians be aware of such a condition because early recognition and treatment are crucial to decrease morbidity and mortality.

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Section: Discussionmentioning

confidence: 99%

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Gabi

Milhem

Tovar

et al. 2018

Journal of the Endocrine Society

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“…Among 53 adrenal pheochromocytoma cases, 48 were solitary tumors with a left-to-right ratio of 30:18, and five cases were bilateral. Of these five cases, one was conclusively diagnosed as multiple endocrine neoplasia type 2A (MEN2A) [21]. Our findings indicate that female individuals had a significantly higher predisposition for developing ACTH-producing pheochromocytoma/paraganglioma, constituting 73.1% (49 out of 67 cases) of all cases.…”

Section: Discussionmentioning

confidence: 76%

Pheochromocytoma With High Adrenocorticotropic Hormone Production Capacity Without Pigmentation and Cushingoid Symptoms: A Case Report With a Literature Review

Mizutani,

Isshiki,

Shimizu

et al. 2024

Cureus

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Pheochromocytoma or paraganglioma (PPGL) originating from chromaffin cells can produce diverse hormones in addition to catecholamines, including adrenocorticotropic hormone (ACTH). In pheochromocytoma, high levels of ACTH might not result in pigmentation as typically observed in Addison's disease, and patients might not exhibit the symptoms of Cushing's syndrome, despite ACTHdependent hypercortisolism. A 63-year-old male patient with hypertension was admitted to our facility, and computed tomography (CT) revealed a large right adrenal tumor. Despite high plasma ACTH (700-1300 pg/mL) and serum cortisol (90-100 µg/dL) levels, no physical pigmentation or Cushingoid symptoms were observed. Urinary metanephrine and normetanephrine levels reached as high as 16.0 mg and 3.2 mg, respectively. 123 I-metaiodobenzylguanidine (MIBG) scintigraphy was negative. Low-dose dexamethasone paradoxically increased ACTH and cortisol levels, indicating the potential positive feedback regulation of both hormones by glucocorticoids. The patient was diagnosed with an ACTH-producing pheochromocytoma and underwent successful laparoscopic surgery to remove the adrenal tumor under the intravenous administration of a high-dose α-blocker and hydrocortisone. The levels of ACTH, cortisol, and urinary metanephrine/normetanephrine returned close to normal after tumor removal. We report a rare case of pheochromocytoma with extremely high ACTH/cortisol production but without pigmentation or Cushingoid symptoms. We also reviewed previous reports of ACTH-producing PPGL regarding the paradoxical regulation of ACTH/cortisol by glucocorticoids, pigmentation, Cushingoid symptoms, and negativity of 123 I-MIBG scintigraphy.

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“…The most common causes of ectopic ACTH syndrome are malignancies that include small cell lung cancer (SCLC), bronchial carcinoids, islet cell tumors of the pancreas, thymic carcinoids, and medullary thyroid cancer. ACTH-producing pheochromocytoma is one cause of ectopic ACTH syndrome, which has been reported to range from 2% to 25% [2][3][4][5][6][7][8], fewer than 30 cases globally and only 3 cases have been reported in Korea [9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma presented as Cushing syndrome and complicated by invasive aspergillosis

Cho

Jeong

Lee

et al. 2015

Yeungnam Univ J Med

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Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma has been rarely reported, whereas only a few cases of Cushing syndrome accompanied by opportunistic infections have been reported. We experienced a patient with pheochromocytoma with ectopic Cushing syndrome complicated by invasive aspergillosis. A 35-year-old woman presented with typical Cushingoid features. Her basal plasma cortisol, ACTH, and 24-hour urine free cortisol levels were significantly high, and 24-hour urine metanephrine and catecholamine levels were slightly elevated. The endogeneous cortisol secretion was not suppressed by either low-or high-dose dexamethasone. Abdominal computed tomography (CT) revealed a heterogeneous enhancing mass measuring approximately 2.5 cm in size in the left adrenal gland. No definitive mass lesion was observed on sellar magnetic resonance imaging. On fluorine-18 fluorodeoxyglucose positron emission tomography/CT, a hypermetabolic nodule was observed in the left upper lung. Thus, we performed a percutaneous needle biopsy, which revealed inflammation, not malignancy. Thereafter, we performed a laparoscopic left adrenalectomy, and its pathologic finding was a pheochromocytoma with positive immunohistostaining for ACTH. After surgery, the biochemistry was normalized, but the clinical course was fatal despite intensive care because of the invasive aspergillosis that included the lungs, retina, and central nervous system.

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Ectopic ACTH Syndrome with Bilateral Pheochromocytoma in Multiple Endocrine Neoplasia Type 2A

Cited by 4 publications

References 19 publications

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Pheochromocytoma With High Adrenocorticotropic Hormone Production Capacity Without Pigmentation and Cushingoid Symptoms: A Case Report With a Literature Review

Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma presented as Cushing syndrome and complicated by invasive aspergillosis

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