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MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli
Background: Adrenocortical carcinoma is a rare, often aggressive tumor that is more common in women than men. The tumors are more often than not biochemically active in adults, and the majority secrete glucocorticoids alone (1,2).Clinical case: A 57yo woman w/ longstanding hypertension and history o...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Endocrine Society
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6550672/ http://dx.doi.org/10.1210/js.2019-MON-323 |
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author | Parham, Johnathon Adamson, Samantha Ostlund, Richard |
author_facet | Parham, Johnathon Adamson, Samantha Ostlund, Richard |
author_sort | Parham, Johnathon |
collection | PubMed |
description | Background: Adrenocortical carcinoma is a rare, often aggressive tumor that is more common in women than men. The tumors are more often than not biochemically active in adults, and the majority secrete glucocorticoids alone (1,2).Clinical case: A 57yo woman w/ longstanding hypertension and history of total abdominal hysterectomy presented with shortness of breath. She reported worsening of her hypertension over the past six months associated with male pattern balding, body hair growth, anxiety, facial and abdominal swelling for the past four months. Computed tomography PE protocol showed bilateral pulmonary emboli and multiple pulmonary nodules. Subsequent computed tomography chest/abdomen/pelvis revealed pulmonary nodules in both lungs, the largest being 3.7cm x 2.5cm in the right upper lobe, and a large right retroperitoneal mass centered at the expected position of the right adrenal gland measures 12.4cm x 10.5cm causing mass effect on the inferior surface of the liver along with bulky mediastinal, hilar, paratracheal, gastrohepatic, portocaval, and retroperitoneal lymphadenopathy. Exam was significant for cushingoid habitus with central obesity, rounded facies, supraclavicular and dorsocervical fat pads, proximal muscle weakness, frontotemporal balding, severe hirsutism consistent with modified ferriman-gallwey score of 13, and an annular, scaly rash along gluteal cleft. 8am serum cortisol was 65mcg/dL. 24 hour urinary cortisol was 450mcg/24 hour. ACTH was undetectable <2.0pg/mL. Total Testosterone was 835ng/dL. DHEAS was 1,000mcg/dL. Renin, aldosterone, plasma fractionated metanephrines were normal. Excisional supraclavicular lymph node biopsy was positive for metastatic adrenocortical carcinoma. Subsequent FDG PET/CT also showed metastatic disease in the liver, right humeral head, sacrum, and bilateral iliac bones. Punch biopsy of the rash was consistent with tinea cruris. The patient was moved to the oncology service and started on therapy with mitotane, cisplatin, doxorubicin and etoposide.Conclusion: Rapid virilization is concerning for an underlying malignancy and should always include androgen evaluation with a high index of suspicion for ovarian or adrenal tumors.References:1. Wajchenberg BL, Albergaria Pereira MA, Medonca BB, et. Al. Adrenocortical carcinoma: clinical and laboratory observations. Cancer. 2000;88(4):711.2. Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. 2003;169(1):5. |
format | Online Article Text |
id | pubmed-6550672 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Endocrine Society |
record_format | MEDLINE/PubMed |
spelling | pubmed-65506722019-06-13 MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli Parham, Johnathon Adamson, Samantha Ostlund, Richard J Endocr Soc Tumor Biology Background: Adrenocortical carcinoma is a rare, often aggressive tumor that is more common in women than men. The tumors are more often than not biochemically active in adults, and the majority secrete glucocorticoids alone (1,2).Clinical case: A 57yo woman w/ longstanding hypertension and history of total abdominal hysterectomy presented with shortness of breath. She reported worsening of her hypertension over the past six months associated with male pattern balding, body hair growth, anxiety, facial and abdominal swelling for the past four months. Computed tomography PE protocol showed bilateral pulmonary emboli and multiple pulmonary nodules. Subsequent computed tomography chest/abdomen/pelvis revealed pulmonary nodules in both lungs, the largest being 3.7cm x 2.5cm in the right upper lobe, and a large right retroperitoneal mass centered at the expected position of the right adrenal gland measures 12.4cm x 10.5cm causing mass effect on the inferior surface of the liver along with bulky mediastinal, hilar, paratracheal, gastrohepatic, portocaval, and retroperitoneal lymphadenopathy. Exam was significant for cushingoid habitus with central obesity, rounded facies, supraclavicular and dorsocervical fat pads, proximal muscle weakness, frontotemporal balding, severe hirsutism consistent with modified ferriman-gallwey score of 13, and an annular, scaly rash along gluteal cleft. 8am serum cortisol was 65mcg/dL. 24 hour urinary cortisol was 450mcg/24 hour. ACTH was undetectable <2.0pg/mL. Total Testosterone was 835ng/dL. DHEAS was 1,000mcg/dL. Renin, aldosterone, plasma fractionated metanephrines were normal. Excisional supraclavicular lymph node biopsy was positive for metastatic adrenocortical carcinoma. Subsequent FDG PET/CT also showed metastatic disease in the liver, right humeral head, sacrum, and bilateral iliac bones. Punch biopsy of the rash was consistent with tinea cruris. The patient was moved to the oncology service and started on therapy with mitotane, cisplatin, doxorubicin and etoposide.Conclusion: Rapid virilization is concerning for an underlying malignancy and should always include androgen evaluation with a high index of suspicion for ovarian or adrenal tumors.References:1. Wajchenberg BL, Albergaria Pereira MA, Medonca BB, et. Al. Adrenocortical carcinoma: clinical and laboratory observations. Cancer. 2000;88(4):711.2. Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. 2003;169(1):5. Endocrine Society 2019-04-30 /pmc/articles/PMC6550672/ http://dx.doi.org/10.1210/js.2019-MON-323 Text en Copyright © 2019 Endocrine Society https://creativecommons.org/licenses/by-nc-nd/4.0/ This article has been published under the terms of the Creative Commons Attribution Non-Commercial, No-Derivatives License (CC BY-NC-ND; https://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Tumor Biology Parham, Johnathon Adamson, Samantha Ostlund, Richard MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli |
title | MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli |
title_full | MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli |
title_fullStr | MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli |
title_full_unstemmed | MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli |
title_short | MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli |
title_sort | mon-323 metastatic adrenocortical carcinoma presenting with mixed cushing's and virilization syndrome along with bilateral pulmonary emboli |
topic | Tumor Biology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6550672/ http://dx.doi.org/10.1210/js.2019-MON-323 |
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