Pheochromocytoma arising from an ectopic adrenal tissue in multiple endocrine neoplasia type 2A

Ohsugi, Haruyuki; Takizawa, Nae; Kinoshita, Hidefumi; Matsuda, Tadashi

doi:10.1530/edm-19-0073

Cited by 4 publications

(7 citation statements)

References 10 publications

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“…Both cortex and medulla can be present in the ectopic adrenal tissue, if the separation takes place in a later phase during organogenesis, after the neural crest's migra- tion into the cortex. Usually, the medulla might be found in the ectopic tissue when the separation takes place near the location of the normal adrenal glands [7,[12][13][14][15]. Pheochromocytoma can be sporadic or hereditary.…”

Section: Discussionmentioning

confidence: 99%

“…Korpershoek et al showed that MEN2-associated pheochromocytoma exhibits genomic alterations identical to adrenal medullary hyperplasia in MEN2 syndrome, suggesting that adrenal medullary hyperplasia could represent a precursor lesion for pheochromocytoma [13]. Recently, Ohsugi et al described a case of bilateral adrenal pheochromocytoma associated with ectopic adrenal medullary hyperplasia that 12 months later developed into a pheochromocytoma, in a patient with MEN2A syndrome [14]. All patients with a diagnosis of pheochromocytoma should be considered for genetic screening [6,16].…”

Section: Discussionmentioning

confidence: 99%

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Unusual case of infrarenal pheochromocytoma developed on ectopic adrenal tissue: An autopsy case report

Chiciudean¹,

Jung

Bara³

et al. 2022

Acta Marisiensis - Seria Medica

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Introduction: Pheochromocytomas are catecholamine-secreting tumors arising from the chromaffin cells of the adrenal medulla. We describe an unusual case of pheochromocytoma located in the infrarenal area and associated with bilateral adrenal hyperplasia. Case presentation: A 70-year-old patient was admitted for syncope, diaphoresis, and high blood pressure. Computed tomography showed a 73×70×72 mm retroperitoneal mass of the left infrarenal area. High levels of metanephrine and noradrenaline were found in the urine. A tumor resection was performed. A few days after surgery, the blood pressure suddenly decreased and could not be restored, resulting in the death of the patient. Histopathological examination of the surgical specimen revealed a proliferation of monotonous cells, with eosinophilic cytoplasm, round nuclei with prominent nucleoli arranged in clusters, and capsular and vascular invasion. The tumor cells expressed synaptophysin and chromogranin, without positivity for inhibin A or S100 protein. At the autopsy, both adrenal glands showed hyperplasia but unrelated to the tumor mass. The histological aspect, location, and immunophenotype indicated an ectopic infrarenal pheochromocytoma. Conclusions: In patients with pheochromocytoma developed on ectopic adrenal tissue, clinical management might be difficult, and diagnosis can be sometimes established only based on post-mortem histopathological examination. Autopsy can be extremely useful in such cases with unexplained evolution.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Unusual case of infrarenal pheochromocytoma developed on ectopic adrenal tissue: An autopsy case report

Chiciudean¹,

Jung

Bara³

et al. 2022

Acta Marisiensis - Seria Medica

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“…This interaction between adrenal chromaffin cells and cortex is essential for the maturation of adrenal medulla, whereas the Zuckerkandl organ, the largest extra-adrenal chromaffin accumulation in mammals, lacks such interaction with steroidogenic tissue and undergoes involution after birth (postnatal autophagy-mediated cell death) in the absence of cortex-derived glucocorticoids ( 14 ). This appears to be the reason for the absence of literature describing ectopia of adrenal medulla only, that is not surrounded by cortex, while entities related to adrenal or extra-adrenal chromaffin cells (pheochromocytoma and paraganglioma), and even pheochromocytoma in ectopic adrenal gland have been reported ( 15 , 16 ).…”

Section: Ectopic Adrenal Tissues Contain Either Cortex Only or Both C...mentioning

confidence: 99%

Emergence of Ectopic Adrenal Tissues-What are the Probable Mechanisms?

Tarçın

Ercan

2022

Jcrpe

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Ectopic adrenal tissue, defined as the formation of adrenal tissue in an abnormal anatomical location, is not a rare entity and may have clinical significance. Even though the mechanism for their emergence has not been fully understood, numerous cases of ectopic adrenal tissue have been reported, mostly in the vicinity of the original location of adrenal gland, such as in kidneys and gonads. In these cases, most authors attributed their emergence to a probable migration defect. However, this mechanism does not simply explain the ectopic tissues in remote locations, such as in the hypophysis or lungs. This review summarizes these reports, describing many different locations in which ectopic adrenal tissues were encountered, together with their suggested mechanisms.

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“…In another case, pheochromocytoma arising from ectopic adrenal tissue was presented in a 21-year-old woman with multiple endocrine neoplasia type 2A showing that, not only the cortex but also the medulla can be found in ectopic adrenal tissue, even leading to a neoplasia. 16 According to data, functional hypertrophy may develop in ectopic adrenal tissue after removal or destruction of normal adrenal glands. 1 Also, cases of Cushing's syndrome or primary aldosteronism arising from ectopic tissue located in the appendix may mimic carcinoid tumor.…”

Section: Cases In Womenmentioning

confidence: 99%

Clinicopathological Evaluation of 15 Ectopic Adrenal Tissues

et al. 2021

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Background: Ectopic adrenal tissue is a rare condition associated with embryological development defects seen in many different areas in the abdomen and pelvis. Here, we aimed to discuss the clinicopathological features of ectopic adrenal tissues diagnosed in our clinic, in light of the literature. Methods: We included cases of ectopic adrenal tissues incidentally detected in the specimens from patients undergoing operation for various diagnoses during 2012-March 2020. The cases were examined according to gender, age, location and accompanying pathological diagnoses. During this period, 15 cases of ectopic adrenal tissues (6 paratubal, 3 paraovarian, 2 paratesticular, 1 spermatic cord, 1 paraaortic, 1 liver capsule, 1 omentum) were detected accompanied by two endometrial carcinomas, two serous cystadenomas, one seminoma, one mixed germ cell tumor, one bilateral ovarian serous carcinoma and hepatic high-grade colon adenocarcinoma metastasis. Results: In this report, the fifth ectopic adrenal tissue accompanying a malignant testicular tumor, the fifth and sixth ectopic adrenal tissues occurring with ovarian serous cystadenoma, the first case observed with serous cystadenocarcinoma and the first case detected with hepatic adenocarcinoma metastasis are presented. Our cases are mostly women and adult men. Conclusion: Ectopic adrenal tissues can lead to hormonal problems and also to adrenal cortex and medulla neoplasms. Microscopically, they may be confused with clear cell gynecological and germ cell tumors. If the ectopic focus is misdiagnosed as an implant, a benign entity may be incorrectly reported as malignant. Also, it is important to prevent mis-staging in malignancies. For precise diagnosis, an immunopanel such as inhibin, melan A, and calretinin can be performed.

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Pheochromocytoma arising from an ectopic adrenal tissue in multiple endocrine neoplasia type 2A

Cited by 4 publications

References 10 publications

Unusual case of infrarenal pheochromocytoma developed on ectopic adrenal tissue: An autopsy case report

Unusual case of infrarenal pheochromocytoma developed on ectopic adrenal tissue: An autopsy case report

Emergence of Ectopic Adrenal Tissues-What are the Probable Mechanisms?

Clinicopathological Evaluation of 15 Ectopic Adrenal Tissues

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