Adrenocortical carcinoma masquerading as pheochromocytoma: a histopathologic dilemma
| Autor: | Karel Pacak, Impana Shetty, Karlyne M. Reilly, Jaydira Del Rivero, Maria Merino, Sarah N. Fuller, Barbara J. Thomas, Margarita Raygada, Brigitte C. Widemann |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
Adrenocortical carcinoma
Sandostatin Endocrinology Diabetes and Metabolism White SF-1 January Ultrasound scan Haematoxylin and eosin staining lcsh:Diseases of the endocrine glands. Clinical endocrinology 0302 clinical medicine Pathology Gallium scan Mitotane Anthracyclines Adrenal Etoposide Molecular pathology Headache Pembrolizumab Adrenalectomy Vincristine Immunohistochemistry Lynch syndrome Dacarbazine Oncology 030220 oncology & carcinogenesis Hepatocellular carcinoma Hypertension Female Radiology medicine.drug MRI Adult CT scan medicine.medical_specialty Synaptophysin Histopathology 030209 endocrinology & metabolism behavioral disciplines and activities KI-67 Pheochromocytoma 03 medical and health sciences Internal Medicine medicine Flushing Genetics Adrenal adenoma Vimentin Lynch Syndrome lcsh:RC648-665 business.industry Statins Calretinin Endoscopy PET scan medicine.disease Cyclophosphamide Unique/Unexpected Symptoms or Presentations of a Disease United States nervous system Doxorubicin Asthenia Thorascopic surgery Differential diagnosis Cisplatin business Resection of tumour |
| Zdroj: | Endocrinology, Diabetes & Metabolism Case Reports, Vol 1, Iss 1, Pp 1-6 (2020) Endocrinology, Diabetes & Metabolism Case Reports |
| ISSN: | 2052-0573 |
| Popis: | Summary Adrenocortical carcinoma (ACC) is an aggressive cancer that originates in the cortex of the adrenal gland and generally has a poor prognosis. ACC is rare but can be more commonly seen in those with cancer predisposition syndromes (e.g. Li-Fraumeni and Lynch Syndrome). The diagnosis of ACC is sometimes uncertain and it requires the use of precise molecular pathology; the differential diagnosis includes pheochromocytoma, adrenal adenoma, renal carcinoma, or hepatocellular carcinoma. We describe a case of a 57-year-old woman with Lynch Syndrome and metastatic ACC who was initially diagnosed as having pheochromocytoma. The tumor was first identified at 51 years of age by ultrasound followed by a CT scan. She underwent a left adrenalectomy, and the histopathology identified pheochromocytoma. Two years later, she had tumor recurrence with imaging studies showing multiple lung nodules. Following a wedge resection by video-assisted thoracoscopic surgery (VATS), histopathology was read as metastatic pheochromocytoma at one institution and metastatic ACC at another institution. She later presented to the National Institutes of Health (NIH) where the diagnosis of ACC was confirmed. Following her ACC diagnosis, she was treated with mitotane and pembrolizumab which were stopped due to side effects and progression of disease. She is currently receiving etoposide, doxorubicin, and cisplatin (EDP). This case highlights the importance of using a multi-disciplinary approach in patient care. Thorough evaluation of the tumor’s pathology and analysis of the patient’s genetic profile are necessary to obtain the correct diagnosis for the patient and can significantly influence the course of treatment. Learning points: Making the diagnosis of ACC can be difficult as the differential diagnosis includes pheochromocytoma, adrenal adenoma, renal carcinoma, or hepatocellular carcinoma. Patients with Lynch Syndrome should undergo surveillance for ACC as there is evidence of an association between Lynch Syndrome and ACC. Conducting a complete tumor immunoprofile and obtaining a second opinion is very important in cases of suspected ACC in order to confirm the proper diagnosis. A multi-disciplinary approach including genetic testing and a thorough evaluation of the tumor’s pathology is imperative to ensuring that the patient receives an accurate diagnosis and the appropriate treatment. |
| Databáze: | OpenAIRE |
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