CLINICAL IMAGE
Cushing syndrome and bone metastases as the manifestation of adrenocortical carcinoma
Anna Brona, Aleksandra Jawiarczyk-Przybyłowska, Eliza Kubicka, Anna Bohdanowicz-Pawlak, Marek Bolanowski, Joanna Syrycka
Department of Endocrinology, Diabetes and Isotope Therapy, Wroclaw Medical University, Wrocław, Poland
A 61-year-old woman was referred to an ortho- pedic clinic because of severe nontraumatic back pain. Computed tomography (CT) of the lumbo- sacral spine revealed vertebral body compres- sion fractures in Th11 through L4, bone loss, and right adrenal tumor. Treatment with buprenor- phine was initiated and the patient started us- ing an orthopedic corset and a wheelchair. Ad- ditionally, the patient presented symptoms of Cushing syndrome (CS), that is, abdominal obe- sity with thin limbs, a round red face, thin skin, hypertension, diabetes requiring insulin thera- py, without hirsutism.
Clinical symptoms and laboratory test re- sults (increased cortisol levels without typi- cal circadian rhythm at 26.1 µg/dl at 6 AM and 29 µg/dl at 12 PM; decreased adrenocorticotrop- ic hormone levels [<5 pg/ml]; and hypokalemia at 3.1 mmol/l [reference range, 3.8-5 mmol/1]) confirmed adrenocorticotropic hormone-inde- pendent CS. Abdominal CT detected a large right adrenal tumor, 60 × 47 × 51 mm in size, with het- erogeneous contrast enhancement, delayed con- trast washout, and vertebral body compression
fractures (FIGURE 1A). Dual-energy X-ray absorpti- ometry of the lumbar spine and the femoral neck revealed low bone mass.
The patient was referred to the surgery de- partment for adrenalectomy. The histological examination was remarkable for adenomatous hyperplasia of the adrenal cortex. Directly af- ter surgery, clinically and hormonally persistent hypercortisolemia was observed. Thus, follow- -up abdominal and thoracic CT scans were per- formed. An irregular, hypodense lesion without contrast enhancement, 51 × 28 × 26 mm in size, at the postadrenalectomy site and osteolytic le- sions in the thoracic vertebral body were visu- alized (FIGURE 1B). As the patient refused another surgery, metyrapone therapy was started and her condition improved. The third abdominal CT scan (performed after 4 weeks) presented the previ- ously seen soft tissue lesion, with possible inva- sion of the surrounding tissues yet without any abnormalities in the lymph nodes, and multiple osteolytic lesions in bones. The second histologi- cal examination also did not confirm adrenocor- tical carcinoma (ACC) (Weiss score, 2/9). Due to
Correspondence to:
Joanna Syrycka, MD, PHD, Department of Endocrinology, Diabetes and Isotope Therapy, Wroclaw Medical University, ul. Wybrzeże L. Pasteura 4, 50-367 Wrocław, Poland, phone: +48 717 842432, email: joanna.syrycka@umed.wroc.pl
Received: January 6, 2021.
Revision accepted:
February 14, 2021.
Published online: February 24, 2021.
Pol Arch Intern Med. 2021; 131 (4): 384-386
doi:10.20452/pamw.15829 Copyright by the Author(s), 2021
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inconclusive histological findings and the rarity of bone metastases in ACC, 18F-fluorodeoxyglu- cose positron emission tomography-CT (18FDG- -PET/CT) was performed to search for another malignancy. The 18FDG uptake was observed in the postoperative site lesion (maximum stan- dardized uptake value, 3.1) and generalized osteo- lytic skeletal lesions (maximum standardized up- take value, 5.2) (FIGURE 1C-1H). It did not reveal any metastases to the lymph nodes, lungs, and liver or any other malignancy. Diagnostic workup find- ings were strongly suggestive of ACC with bone metastases, which was further confirmed by sur- gical adrenal biopsy. Due to the apparently met- astatic disease, mitotane therapy was initiated.
Unfortunately, despite treatment, the patient died soon.
Adrenocortical carcinoma is a very rare condi- tion (0.7-2 per 1000 000 per year). About 50% to 60% of patients have clinical hormone excess, predominantly CS.1 Bone loss in CS leads to frac- tures in approximately 30% to 76% of patients, especially at the vertebral site.2 In such cases, CT makes the recognition of bone metastases diffi- cult. Adrenocortical carcinoma is usually meta- static at the time of diagnosis. The lung and liver are the most common metastatic sites.1 Bone me- tastases are noted in up to 14% of patients with advanced ACC.3,4 The bone is the single metastat- ic site in only 9% of those patients.3
Although CT and magnetic resonance im- aging represent basic modalities to evaluate the stage of ACC and enable adequate treatment, 18FDG-PET/CT can help to localize all metastases. Nevertheless, despite typical clinical and radio- logical features, obtaining the histological con- firmation of ACC remains a challenge and leads to delay in chemotherapy initiation.
ARTICLE INFORMATION
CONFLICT OF INTEREST None declared.
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HOW TO CITE Brona A, Jawiarczyk-Przybyłowska A, Kubicka E, et al. Cushing syndrome and bone metastases as the manifestation of adrenocor- tical carcinoma. Pol Arch Intern Med. 2021; 131: 384-386. doi:10.20452/ pamw.15829
REFERENCES
1 Fassnacht M, Dekkers OM, Else T, et al. European Society of Endocrinol- ogy Clinical Practice Guidelines on the management of adrenocortical carci- noma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2018; 179: G1-G46.
2 van der Eerden AW, den Heijer M, Oyen WJ, et al. Cushing’s syndrome and bone mineral density: lowest Z scores in young patients. Neth J Med. 2007; 65: 137-141.
3 Berruti A, Libè R, Laganà M, et al. Morbidity and mortality of bone me- tastases in advanced adrenocortical carcinoma: a multicenter retrospective study. Eur J Endocrinol. 2019; 180: 311-320.
4 Libé R, Borget I, Ronchi CL, et al; ENSAT network. Prognostic fac- tors in stage III-IV adrenocortical carcinomas (ACC): an European Net- work for the Study of Adrenal Tumor (ENSAT) study. Ann Oncol. 2015; 26: 2119-2125.