A rare case of right shoulder pain
Asha Bansari, Nila S Radhakrishnan İD , Juan Nicolas Kattan
Medicine, University of Florida Health, Gainesville, Florida, USA
Correspondence to Dr Asha Bansari; asha.bansari@medicine.ufl.edu
Accepted 23 December 2020
SUMMARY
We present a case of a 19-year-old man with right shoulder pain lasting for several months. Abdominal imaging revealed a right adrenal mass directly invading vascular structures into the right atrium. Widespread metastatic adrenocortical carcinoma was confirmed on biopsy. He opted for palliative mitotane treatment with home hospice care. This case emphasises the importance of considering abdominal masses in the differential diagnosis of persistent right shoulder pain after common causes have been ruled out. Early diagnosis could be potentially life-saving.
BACKGROUND
An unusual presentation of a rare disease: This is a case of functional adrenocortical carcinoma extending through the inferior vena cava (IVC) to the right atrium (RA) presenting as right shoulder pain.
Adrenocortical carcinomas are rare malignant neoplasms with a prevalence of 0.5-2 cases per million people per year. Usual metastatic sites are the lung, lymph node, liver and bone. Invasion through the IVC to the RA is very rare.1-8 Nearly half the patients with adrenocortical carcinoma present with a clinical syndrome of excess hormone secretion of either glucocorticoids or androgens.9 Patients with non-functioning tumours present with symptoms due to local growth and compression of surrounding structures. Our patient presented with compression and extension symptoms despite having a hormone-producing tumour.
CASE PRESENTATION
A 19-year-old man presented with right shoulder pain for several months with symptoms worsening over 5 days preceding his initial emergency room visit. The patient reported right shoulder pain for which he had been seeing a chiropractor for 4-6 months. He denied fevers, chills, nausea, vomiting, diarrhoea, night sweats or significant weight loss. The pain progressed over time to include the right upper quadrant (RUQ). Physical examination was significant for RUQ tenderness and abdominal distention with a palpable abdominal mass. Pitting oedema was observed on both lower extremities along with scrotal oedema.
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To cite: Bansari A, Radhakrishnan NS, Kattan JN. BMJ Case Rep 2021;14:e232266. doi: 10.1136/bcr-2019- 232266
INVESTIGATIONS
CT of the abdomen (figures 1 and 2) showed a large right adrenal mass of 11.2×11.9x13.5 cm with areas of enhancement, hypodensity and periph- eral calcification. This mass invaded the IVC and hepatic veins and extended into the RA. The mass
pushed the right kidney down, laterally, and invagi- nated up into the inferior aspect of the liver.
DIFFERENTIAL DIAGNOSIS
Pheochromocytoma was ruled out after endocrine workup showed only slight elevations of 24-hour urine normetanephrines (less than two times the upper limit of normal) as well as plasma norme- tanephrines (1.2 nmol/L). The majority of patients with pheochromocytoma are shown to have plasma normetanephrine concentrations of more than 2.2 nmol/L. Plasma metanephrine level in our patient was within normal limits (0.13 nmol/L). Chromogranin A was within normal range ruling out a neuroendocrine origin for his tumour.
ABDIPLL
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Case report
Aldosterone and renin levels as well as aldosterone/renin activity were within normal range. There was no evidence of multiple endocrine neoplasia syndrome (no hypercalcemia and normal parathyroid hormone levels and calcitonin levels). Dehydroepi- androsterone was normal at 166 µg/dL.
A dexamethasone suppression test showed no suppression of cortisol (level: 9.4 µg/dL) consistent with autonomous produc- tion of cortisol by the tumour.
TREATMENT
The patient opted for palliative mitotane therapy. Mitotane is an insecticide derivative that produces adrenocortical necrosis by inhibiting corticoid biosynthesis and destroying adrenocortical cells.10 11 At low plasma concentrations, mitotane suppresses secretion of adrenal steroids, providing symptomatic improve- ment and partial regression of the endocrine dysfunction expe- rienced in patients with functioning tumours.11 In patients with advanced disease, objective response may be obtained in approx- imately 20%-30% of cases using mitotane alone.
OUTCOME AND FOLLOW-UP
The patient was discharged home with hospice. Unfortunately, he passed within 4 months of his diagnosis.
DISCUSSION
Adrenocortical carcinoma is a rare disease with a poor prognosis. The disease is more common in women with a female to male ratio of 1.5-2.5:1 and bimodal age distribution ranging from 2
Learning points
Take home message: keep abdominal masses in the differential diagnosis of right shoulder pain.
Right shoulder pain is well known to represent referred pain from liver and gall bladder disease as the phrenic nerve innervates the diaphragm and gall bladder by passing through a foramen on the vena cava at the central tendon of the diaphragm.
The right phrenic nerve runs through the right side of the superior vena cava and right atrium, descends between the pericardium and mediastinum and reaches the diaphragm. The adrenal gland is located in the retroperitoneal space and usually does not impact the phrenic nerve. However, the enlarged adrenal gland or the widespread metastatic extension to the liver, vena cava and right atrium in our patient possibly irritated the right phrenic nerve inducing his right-sided shoulder pain.
to 5 years and 40 to 50 years.12 Most common metastatic sites are the lung, liver and bone.13 Our patient was a 19-year-old man with an unusual presentation of a rare disease leading to delay in diagnosis with distant metastasis. To our knowledge, this is the first case report with such presentation. On literature review, we did not find another case with referred shoulder pain due to phrenic nerve and diaphragmatic excitation by an adreno- cortical carcinoma as the presenting complain. However, pheo- chromocytomas with similar presentation have been reported.14
Contributors AB took care of the patient, assisted with details regarding the case, helped to write and reviewed the manuscript. JNK helped to interpret the data, assisted with figures, helped to write and reviewed the final manuscript. NSR helped to interpret the date, assisted with figures, helped to write and reviewed and edited the final manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
ORCID İD Nila S Radhakrishnan http://orcid.org/0000-0002-7666-356X
REFERENCES
1 Schramek P, Dünser E, Bhargabha A, et al. Adrenal cortical carcinoma: preoperative demonstration of right atrial extension by sonography and computerized tomography. J Urol 1985; 133:260-2.
2 Shahian DM, Nieh PT, Libertino JA. Resection of atriocaval adrenal carcinoma using hypothermic circulatory arrest. Ann Thorac Surg 1989;48:421-2.
3 Galli R, Parlapiano M, Pace Napoleone C, et al. [Neoplastic caval and intracardiac thrombosis secondary to reno-adrenal tumors. One-stage surgical treatment in deep hypothermia and cardiocirculatory arrest]. Minerva Urol Nefrol 1994;46:105-11.
4 Yamana D, Yanagi T, Nanbu I. Intracaval invasion of left adrenal cortical carcinoma extending into the right atrium. Radiat Med 1997;15:327-30.
5 Hedican SP, Marshall FF. Adrenocortical carcinoma with intracaval extension. J Urol 1997;158:2056-61.
6 Salinas Sánchez AS, Segura Martín M, Lorenzo Romero JG, et al. [Adrenal cortex carcinoma with right atrium involvement. Surgery with cardiopulmonary bypass]. Actas Urol Esp 2000;24:590-3.
7 Chesson JP, Theodorescu D. Adrenal tumor with caval extension. Scan J Urol Nephrol 2002;36:71-3.
8 Rosen B, Rozenman Y, Harpaz D. Extension of adrenocortical carcinoma into the right atrium — echocardiographic diagnosis: a case report. Cardiovasc Ultrasound 2003; 1:5.
9 Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 2003; 169:5-11.
10 Paragliola R, Torino F, Papi G. Role of mitotane in Adrenocorticalcarcinoma. Eur endocrinol 2018;09:62-6.
11 Ledbetter D, Johnson P. Endocrine surgery in children. Berlin, Heidelberg: Springer, 2017: 133-8.
12 Okazumi S, Asano T, Ryu M, et al. [Surgical resection of adrenal carcinoma extending into the vena cava, right atrium and ventricle: case report and review of the literature]. Nihon Geka Gakkai Zasshi 1987;88:231-8.
13 Bilge A, Pierard LA, Kulbertus HE. Isolated cardiac metastasis of adrenal carcinoma: transesophageal echocardiographic features. Am Heart J 1996; 132:1066-8.
14 Hagiya H, Nakamura E, Terasaka T, et al. Pheochromocytoma manifesting persistent right shoulder pain and Hypochondralgia. J Gen Fam Med 2015;16:292-6.
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