CLINICAL IMAGE
Adrenocortical carcinoma associated with giant bilateral myelolipomas in classic congenital adrenal hyperplasia
Agnieszka Łebek-Szatańska1, Karolina M. Nowak1, Radosław Samsel2, Katarzyna Roszkowska-Purska3, Wojciech Zgliczyński1, Lucyna Papierska1
1 Department of Endocrinology, Centre of Postgraduate Medical Education, Bielanski Hospital, Warsaw, Poland
2 Division of Surgery, Department of Oncology, Maria Skłodowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland
3 Department of Pathology, Maria Skłodowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland
A 32-year-old male patient, diagnosed with a clas- sic form of congenital adrenal hyperplasia (CAH) due to 21a-hydroxylase deficiency as a neonate was referred to the Department of Endocrinol- ogy in March 2018 because of poor disease con- trol and rapidly enlarging bilateral adrenal masses (FIGURE 1A and 1B). A year and a half before the ad- mission, an adrenal biopsy was performed (in an- other hospital), which revealed a benign tumor, myelolipoma. However, due to the mass effect and heterogenic appearance on imaging, the left side tumor was surgically removed 2 years after its initial presentation. Macroscopic examination revealed a mass of 350 mm in the longest dimen- sion and almost 2 kg of weight (FIGURE 1C). Micro- scopically, the tumor was composed mainly of myelolipoma tissue, with adrenocortical cell “is- lands.” However, when assessed using the Weiss score, these foci were diagnosed not as an adre- nocortical adenoma but adrenocortical carcinoma
(ACC). From among 9 histologic criteria of ma- lignancy, 6 were positive (with 3 necessary to di- agnose ACC), including nuclear grade (Fuhrman’s III), atypical mitotic figures, 10% of clear cells, dif- fuse architecture, and sinusoidal and capsular in- vasion (FIGURE 1D). Therefore, chemotherapy with mitotane was introduced due to a large size of the tumor, previous biopsy, and testicular adrenal rest tumors. The patient currently adheres to rig- orous hormonal treatment and monitoring regi- men, and is awaiting right-sided adrenalectomy.
Myelolipomas are uncommon, benign, and nonfunctioning adrenal masses, composed of mature adipose tissue and hemopoietic elements. They usually require surgical excision only when becoming large and symptomatic.1 However, in very rare cases, collision tumors of 2 or more histologically distinct compounds are encoun- tered, such as myelolipoma coexisting with adre- nocortical adenoma or carcinoma.2 An increased
Correspondence to: Agnieszka Łebek-Szatańska, MD, Department of Endocrinology, Centre of Postgraduate Medical Education, Bielanski Hospital, ul. Cegłowska 80, 01-809 Warszawa, Poland, phone: +48 22569 0529, email: agalebek@gmail.com Received: February 12, 2019. Revision accepted: April 3, 2019. Published online: April 4, 2019. Pol Arch Intern Med. 2019; 129 (7-8): 549-550 doi:10.20452/pamw.14788 Copyright by Medycyna Praktyczna, Kraków 2019
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FIGURE 1 C - polycyclic, solid, encapsulated left adrenal collision tumor of 350×110×90 mm in size and a weight of 1780 g; D - adrenal cortical carcinoma (white arrow) and myelolipoma (black arrow) on light microscopy (hematoxylin and eosin staining; magnification ×200)
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prevalence of adrenal tumors has been report- ed in patients with CAH and poor compliance, due to chronic adrenocorticotropic hormone (ACTH) stimulation, which acts as a growth and metaplasia-inducing factor. However, fewer than 20 cases of giant bilateral myelolipomas in CAH patients have been reported to date, and the role of ACTH or other hormones in their development is still uncertain.3
The presented case highlights the need for proper and careful management of patients with CAH. However, it also shows that the risk of ma- lignancy exists even in tumors considered as be- nign. Adrenocortical carcinoma is a rare neoplasm with low survival rates.4 Histopathologic diagno- sis based on multiparameter scoring systems is of- ten challenging. In general, adrenal tumors should preferably be excised and examined as a whole, thus indications for biopsy are very limited and only to cases in which the diagnosis would change the course of treatment. In our patient, the biopsy was completely misguided. What is more, the sus- picion of ACC constitutes a strong contraindica- tion to biopsy due to the possibility of neoplas- tic cell spread in the needle conduit. Therefore, mitotane treatment was introduced because this type of additional treatment may be associated with better outcome.4 Mitotane has a direct and selective cytotoxic effect on adrenal cortex cells, thereby inducing permanent atrophy of any nor- mal or malignant adrenal tissue that is left after the operation. However, also in patients with CAH who are not candidates for surgery, it may play a role in restoring fertility impaired by tes- ticular adrenal rest tumors.5
ARTICLE INFORMATION
CONFLICT OF INTEREST None declared.
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HOW TO CITE Łebek-Szatańska A, Nowak KM, Samsel R, et al. Adreno- cortical carcinoma associated with giant bilateral myelolipomas in classic congenital adrenal hyperplasia. Pol Arch Intern Med. 2019; 129: 549-550. doi:10.20452/pamw.14788
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