Virilizing Adrenocortical Carcinoma Resected With Negative Margins Treated With Surgery Alone
The American Surgeon 2022, Vol. 88(4) 781-783 C The Author(s) 2021 Article reuse guidelines: sagepub.com/journals-permissions DOI: 10.1177/00031348211050840 journals.sagepub.com/home/asu SAGE
Tyler Fields, MD’ D, Kelsee Felux, BA2, Alejandro Chavarriaga, MD’, Philip Ramsay, MD’, and Evan Weitman, MD3
Abstract
Adrenocortical carcinoma is a rare malignancy. A virilizing adrenocortical carcinoma is even more unique of a diagnosis. In this report, we present a rare case of this uncommon tumor with an interesting presentation, clearly documented physical exam changes over a span of at least 8 years, and a technically challenging case. We also briefly review the management of adrenocortical carcinoma. The tumor was successfully resected with no planned adjuvant treatment at this time. The patient had recurrence of menstrual cycle post-operatively and required no steroid supplementation.
Keywords
adrenal, general surgery, surgical oncology
Discussed in this report is a rare case of a virilizing ad- renocortical carcinoma. At baseline, adrenocortical carci- noma (ACC) is an exceedingly uncommon diagnosis. With an incidence of one to two cases per million people per year, ACC accounts for .02 -. 2% of all related cancer deaths.1 Most ACCs are sporadic with a bimodal distri- bution favoring females over males and more aggressive pathology in older patients.2 With roughly 60% of all ACCs being biologically active, roughly 45% present with Cushing’s syndrome, 25% with mixed Cushing’s and virilization features (excess glucocorticoids and an- drogens), and 10% with virilization alone.3 Glucocorticoid producing tumors can present as weight gain, cushingoid physical features, weakness, or insomnia. Androgen se- creting tumors present as hirsutism, deepening voice, male pattern baldness, and oligomenorrhea.4
The patient is a forty-four-year-old African American woman with a past medical history of hypertension and atopic dermatitis that presented in September 2020 complaining of right-sided abdominal pain and right flank pain. The pain had intermittently occurred over the last 2 years with no memorable time of onset. She also admitted to an unintentional forty-pound weight loss over the last 3 years. Her other pertinent symptoms were decreased appetite and amenorrhea. She had a family history of early menopause and atopic dermatitis, but denied any history of cancer. She has a ten-pack a year tobacco smoker history, daily marijuana use, and occasional
alcohol consumption. On examination, she had a masculine appearance with acne and nodular skin lesions, noticeable facial and truncal hair growth, hyperpigmented skin lesions on her trunk, atrophic labia and breasts, deepened voice, and male pattern baldness (Figure 1). The abdomen was larger and tender with an ill-defined mass in the right upper quadrant (Figure 2).
Endocrinology laboratory work up was relevant for elevated 17-hydroxyprogesterone at 2410 ng/dL (139- 431 ng/dL) and androstenedione elevated at 5149 ng/dL (73-230 ng/dL). The AM cortisol was elevated at 6.6 mcg/ dL (<1.8 mcg/dL) after low dose dexamethasone sup- pression test, DHEA sulfate elevated at 1962 mcg/dL (23- 266 mcg/dL), dopamine elevated at 49 pg/mL (< 10 pg/ mL supine or <95 pg/mL upright), and plasma renin activity decreased at .13 ng/ml/h (.25-5.82 ng/ml/h). Testosterone was elevated at 821 ng/dL (8-48 ng/dL). Tumor markers were negative including HCG <3, CEA
‘Department of Graduate Medical Education, Wellstar Atlanta Medical Center, Atlanta, GA, USA
2Ross University School of Medicine, Miramar, FL, USA
3Piedmont Atlanta Hospital, Atlanta, GA, USA
Corresponding Author:
Tyler Fields, MD, Department of Graduate Medical Education, Wellstar Atlanta Medical Center, 303 Parkway Drive, NE, 4th floor, Atlanta, GA 30312-1212, USA.
Email: tyler.fields@wellstar.org
1.3, Ca 19-9 <3, and Ca 125 11. A pelvic ultrasound showed a non-visualized right ovary with no suspicious adnexal or ovarian mass.
Patient was taken for an open right adrenalectomy in October 2020. Given the size and location of her tumor, it was a technically challenging case. The liver was fully mobilized with complete isolation of the infra hepatic IVC with its branches. Glisson’s capsule was degloved to maintain a negative margin with the tumor capsule re- maining intact. There were multiple parasitic vessels coming directly off the IVC that were ligated. On pathology, the tumor was 21.2 × 16.7 x 14.8 cm with 5 uninvolved lo- coregional lymph nodes and a mitotic rate of <20 mitoses/50 hpf. Vascular invasion was present on the tumor with in- vasion into, but not through, the capsule. Final pathology was stage II adrenocortical carcinoma. Postoperatively, the patient did not require steroids. At her endocrinology follow-
up appointment in December 2020, she noted the return of her menstrual cycle. This was her first cycle since 2018.
A virilizing adrenocortical carcinoma is a rare malig- nancy. Due to this patient’s career, there is video docu- mentation of change in her physical appearance over the span of 8+ years. From 2010 to 2014 to 2018 you can clearly see a progression of her physical appearance and a deepening of her voice. With a 5-year survival of stage 2 ACC around 60% and a 2-year recurrence rate of at least 50%, she is at considerable risk for recurrence.4 The main adjuvant treatment for ACC is mitotane. Most studies for mitotane therapy have not been randomized, are retro- spective, and uncontrolled introducing bias, possible misinterpretation of results, and conflicting results for the therapy.1,2 Her case was presented at our multidisciplinary tumor board; given that her resection had negative margins, and given no clear guideline for treatment of duration, it was felt that mitotane would be better used as treatment for a recurrence should it occur. The plan is hormonal sur- veillance with interval imaging moving forward for recurrence.
In summary, this case highlights a rare subset of ad- renocortical carcinoma. It is important to note that with such a low incidence, these tumors can be inadequately addressed and have challenging areas of management. Physical examination is still an important diagnostic tool- especially in the setting of biologically active tumors.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with re- spect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
ORCID iD Tyler Fields [ https://orcid.org/0000-0002-6493-2128
References
1. Bedrose S, Daher M, Altameemi L, et al. Adjuvant therapy in adrenocortical carcinoma: reflections and future directions. Cancers. 2020;12(2):E508. doi:10.3390/cancers12020508
2. Else T, Kim AC, Sabolch A, et al. Adrenocortical carcinoma. Endocr Rev. 2014;35(2):282-326. doi:10.1210/er.2013-1029
3. Fassnacht M, Libé R, Kroiss M, et al. Adrenocortical car- cinoma: a clinician’s update. Nat Rev Endocrinol. 2011;7(6): 323-335. doi:10.1038/nrendo.2010.235
4. Allolio B, Fassnacht M. Clinical review: adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab. 2006; 91(6):2027-2037. doi:10.1210/jc.2005-2639