Para-ovarian adrenal rest tumors: gynecologic manifestations of untreated congenital adrenal hyperplasia

Sisto, J.; Liu, Fong W.; Geffner, Mitchell E.; Berman, Michael L.

doi:10.1080/09513590.2018.1441399

Cited by 13 publications

(6 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In surgical pathology, most adrenal rests are discovered as incidental microscopic findings in various surgical specimens including kidneys or gonads (particularly, spermatic cord [22], rete testis [23][24][25], epididymis [26], ovary/paraovarian [22,[27][28][29][30][31][32][33], salpinx and parasalpinx [22,34,35] ). A recent surgical series from adults identified ectopic adrenal rests in both males and females with a more frequent Fig.…”

Section: Question 2: Why Do Pathologists Need To Know Ectopic or Hete...mentioning

confidence: 99%

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

et al. 2022

View full text Add to dashboard Cite

The new WHO classification of adrenal cortical proliferations reflects translational advances in the fields of endocrine pathology, oncology and molecular biology. By adopting a question–answer framework, this review highlights advances in knowledge of histological features, ancillary studies, and associated genetic findings that increase the understanding of the adrenal cortex pathologies that are now reflected in the 2022 WHO classification. The pathological correlates of adrenal cortical proliferations include diffuse adrenal cortical hyperplasia, adrenal cortical nodular disease, adrenal cortical adenomas and adrenal cortical carcinomas. Understanding germline susceptibility and the clonal-neoplastic nature of individual adrenal cortical nodules in primary bilateral macronodular adrenal cortical disease, and recognition of the clonal-neoplastic nature of incidentally discovered non-functional subcentimeter benign adrenal cortical nodules has led to redefining the spectrum of adrenal cortical nodular disease. As a consequence, the most significant nomenclature change in the field of adrenal cortical pathology involves the refined classification of adrenal cortical nodular disease which now includes (a) sporadic nodular adrenocortical disease, (b) bilateral micronodular adrenal cortical disease, and (c) bilateral macronodular adrenal cortical disease (formerly known primary bilateral macronodular adrenal cortical hyperplasia). This group of clinicopathological entities are reflected in functional adrenal cortical pathologies. Aldosterone producing cortical lesions can be unifocal or multifocal, and may be bilateral with no imaging-detected nodule(s). Furthermore, not all grossly or radiologically identified adrenal cortical lesions may be the source of aldosterone excess. For this reason, the new WHO classification endorses the nomenclature of the HISTALDO classification which uses CYP11B2 immunohistochemistry to identify functional sites of aldosterone production to help predict the risk of bilateral disease in primary aldosteronism. Adrenal cortical carcinomas are subtyped based on their morphological features to include conventional, oncocytic, myxoid, and sarcomatoid subtypes. Although the classic histopathologic criteria for diagnosing adrenal cortical carcinomas have not changed, the 2022 WHO classification underscores the diagnostic and prognostic impact of angioinvasion (vascular invasion) in these tumors. Microscopic angioinvasion is defined as tumor cells invading through a vessel wall and forming a thrombus/fibrin-tumor complex or intravascular tumor cells admixed with platelet thrombus/fibrin. In addition to well-established Weiss and modified Weiss scoring systems, the new WHO classification also expands on the use of other multiparameter diagnostic algorithms (reticulin algorithm, Lin–Weiss–Bisceglia system, and Helsinki scoring system) to assist the workup of adrenal cortical neoplasms in adults. Accordingly, conventional carcinomas can be assessed using all multiparameter diagnostic scheme...

show abstract

Section: Question 2: Why Do Pathologists Need To Know Ectopic or Hete...mentioning

confidence: 99%

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

et al. 2022

View full text Add to dashboard Cite

show abstract

“…No guidelines for the diagnosis or management of OARTs exists. We found only 17 case reports of OARTS in English language literature (13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27), documented in patients with CAH and various enzymatic deficiencies and patients with Nelson syndrome after bilateral adrenalectomy due to Cushing's disease (Table 1). Poor adherence to treatment and highly elevated ACTH levels were hallmarks of these cases, all but one of which were treated with surgery.…”

Section: Discussionmentioning

confidence: 99%

Ovarian adrenal rest tumor in congenital adrenal hyperplasia: Is medical treatment the first line option?

Koren¹,

Koren²,

Khashper³

et al. 2021

Archives of Endocrinology and Metabolism

View full text Add to dashboard Cite

Ovarian adrenal rest tumors (OARTs) are very rare. We describe a case of a young woman with uncontrolled classical congenital adrenal hyperplasia (CCAH), presenting with bilateral OARTs, successfully treated with steroid replacement. A 20-year-old woman, known to have 21OH-CCAH, presented with severe abdominal pain, vomiting, diarrhea, and fever. As a result of poor compliance, 6 months before her admission hirsutism worsened and amenorrhea, hyperpigmentation, and weakness developed. ACTH levels were 278 < pmol/L and 17OHP 91.3 nmol/L. She was admitted for parenteral antibiotics and high-dose hydrocortisone treatment. CT revealed bilateral juxta-ovarian masses (6.2 x 3.6 x 7.4 cm left and 5 x 2.2 x 3.2 cm right) that on MRI were iso-intense in T1 and hypointense in T2, with early enhancement and rapid washout. One week of high-dose hydrocortisone resulted in significant clinical and laboratory improvement and the patient was discharged with 2 mg dexamethasone/day. One month later US revealed shrinkage of the masses and dexamethasone dose was decreased. At three months from discharge, she has resumed regular menses, and a repeated MRI revealed the para-ovarian masses have shrunk. One year after the diagnosis, the para-ovarian masses have shrunk more to 2.8 x 1.9 x 4.3 on the left and 2.1 x 0.9 x 1.2 on the right with less contrast enhancement in comparison to previous test possibly due to fibrotic changes of the tissue. OARTs are rare tumors with a poorly known natural history, and surgery has been the first option in the few reported cases. We demonstrate that medical treatment is a good alternative, leading to significant tumor shrinkage over a short period.

show abstract

“…The role of ACTH is supported by the finding that early-stage TART can regress with high-dose glucocorticoid therapy. Furthermore, TART can be present in males with poorly controlled Addison disease [18] and in the ovaries and/or para-ovarian regions in females with poorly controlled CAH [19]. Further research on the presence of TART in ectopic ACTH syndromes (small cell lung cancers, islet cell tumors, etc.)…”

Section: Discussion/conclusionmentioning

confidence: 99%

Absence of Testicular Adrenal Rest Tumors in Newborns, Infants, and Toddlers with Classical Congenital Adrenal Hyperplasia

Kim

Koppin

et al. 2019

Preprint

Self Cite

View full text Add to dashboard Cite

INTRODUCTION: Testicular adrenal rest tumors (TART) are a known consequence for males with classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. TART are associated with potential infertility in adults. However, little is known about TART in very young males with CAH. OBJECTIVE: We assessed the prevalence of TART in newborn, infant, and toddler males with classical CAH via scrotal ultrasound. METHODS: Males with CAH had scrotal ultrasounds during the first 4 years of life, evaluating testes for morphology, blood flow, and presence of TART. Newborn screen 17-hydroxyprogesterone (17-OHP) and serum 17-OHP at the time of ultrasound were recorded. Bone ages were considered very advanced if ≥ 2SD above chronological age. RESULTS: Thirty-one ultrasounds in 16 males were performed. An initial ultrasound was obtained in four newborns at diagnosis (6.8 ± 2.1 days), six infants (2.2 ± 0.9 months), and six toddlers (2.4 ± 0.9 years). Eleven males had at least one repeat ultrasound. A large proportion (11/16) were in poor hormonal control with an elevated 17-OHP (325 ± 298 nmol/L). One infant was in very poor hormonal control (17-OHP 447 nmol/L) at initial ultrasound, and two toddlers had advanced bone ages (+3.2 and +4.5 SD) representing exposure to postnatal androgens. However, no TART were detected in any subjects. CONCLUSIONS: TART were not found in males up to 4 years of age with classical CAH despite settings with expected high ACTH drive. Further research into the occurrence of TART in CAH may elucidate factors which contribute to the detection and individual predisposition to TART.

show abstract

Para-ovarian adrenal rest tumors: gynecologic manifestations of untreated congenital adrenal hyperplasia

Cited by 13 publications

References 11 publications

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

Ovarian adrenal rest tumor in congenital adrenal hyperplasia: Is medical treatment the first line option?

Absence of Testicular Adrenal Rest Tumors in Newborns, Infants, and Toddlers with Classical Congenital Adrenal Hyperplasia

Contact Info

Product

Resources

About