Learning Overview: After attending this presentation, attendees will have a better understanding of occult pheochromocytoma as a cause of sudden and unexpected death in young patients. Impact Statement: This presentation will impact the forensic science community by presenting the importance of the medicolegal investigation in a case of sudden and unexpected death immediately after a medical procedure. Pheochromocytoma (PHEO or PCC) is a rare catecholamine-producing tumor originating in the adrenal medulla.1 Symptoms are due to catecholamines over-production and related to sympathetic nervous system hyperactivity (i.e., hypertension, tachycardia, sweating, headache, pallor) or a mass effect (i.e., abdominal pain or bowel obstruction). Acute crises can be spontaneous or may be triggered by medications, tumor manipulation, or anesthesiology procedures.2 This study reports the case of a 36-year-old woman admitted to the gynecology department to undergo an abortion at seven weeks of gestational age. Obstetric history was positive for three term pregnancies (two normal spontaneous vaginal deliveries and one caesarean delivery). The patient did not report any illness or medication use. At the preoperative anesthesiology evaluation, vital signs were unremarkable. A medical abortion was performed, followed by Dilation and Curettage (D&C) under epidural anesthesia. The D&C lasted five minutes and immediately after the procedure, the patient developed a severe hypertensive crisis with cardiogenic shock. The patient required intensive resuscitation procedures, including inotropic agents and extracorporeal membrane oxygenation. She subsequently developed hepatic failure that led to coagulopathy. A computed tomography scan revealed a previously unknown left perirenal mass. Eventually, the patient died due to multiorgan failure. An autopsy was ordered to clarify the cause of death and to assess any medical responsibility. At the internal examination, the left mass was found to originate from the adrenal gland; upon gross examination, the cut surface of the mass was diffusely hemorrhagic. Histopathology of samples from the adrenal mass using hematoxylin-eosin stain was suggestive for PHEO.3 The immunohistochemistry analysis on the same samples demonstrated strong and diffuse positivity for chromogranin A in all fields. In addition, a medical inquiry with the woman’s relatives revealed that the deceased was affected with hypertension treated with triple-combination therapy. In the light of these findings, death was ascribed to an acute hypertensive crisis caused by an occult adrenal PHEO. PHEO has been referred to as the great mimic because the symptoms are quite common and sometimes it can be suspected only after paroxysmal episodes such as hypertensive crises. Unmanaged and/or undiagnosed PHEO is dangerous and can lead to serious and potentially lethal cardiovascular complications due to the acute massive secretion of catecholamines.4 In this case, the patient was affected by an undiagnosed PHEO that triggered a lethal hypertensive crisis during a minimally invasive gynecological procedure under local anesthesia. Medical malpractice was excluded because the tumor was occult and the patient did not report her complete medical history. In conclusion, an unknown PHEO can lead to sudden and unexpected death in young patients. In some instances, the diagnosis of PHEO can only be formulated after a complete medicolegal investigation. Reference(s): 1. Harari, A.; Inabnet, W.B. III. Malignant pheochromocytoma: A review. Am J Surg. 2011;201(5):700-708. 2. Bartikoski, S.R.; Reschke, D.J. Pheochromocytoma Crisis in the Emergency Department. Cureus. 2021;13(3):e13683. Published 2021 Mar. 3. Sherwin, R.P. Histopathology of pheochromocytoma. Cancer. 1959; 12:861-877. 4. Naranjo, J.; Dodd, S.; Martin, Y.N. Perioperative Management of Pheochromocytoma. J Cardiothorac Vasc Anesth. 2017;31(4):1427-1439.

Sudden and Unexpected Death After an Induced Abortion: Medical Responsibility or a Hidden Cause?

Lupariello, Francesco;Mirri, Federica;Innocenzi, Fabio;Gabriele, Alessandro;Di Vella, Giancarlo
2022-01-01

Abstract

Learning Overview: After attending this presentation, attendees will have a better understanding of occult pheochromocytoma as a cause of sudden and unexpected death in young patients. Impact Statement: This presentation will impact the forensic science community by presenting the importance of the medicolegal investigation in a case of sudden and unexpected death immediately after a medical procedure. Pheochromocytoma (PHEO or PCC) is a rare catecholamine-producing tumor originating in the adrenal medulla.1 Symptoms are due to catecholamines over-production and related to sympathetic nervous system hyperactivity (i.e., hypertension, tachycardia, sweating, headache, pallor) or a mass effect (i.e., abdominal pain or bowel obstruction). Acute crises can be spontaneous or may be triggered by medications, tumor manipulation, or anesthesiology procedures.2 This study reports the case of a 36-year-old woman admitted to the gynecology department to undergo an abortion at seven weeks of gestational age. Obstetric history was positive for three term pregnancies (two normal spontaneous vaginal deliveries and one caesarean delivery). The patient did not report any illness or medication use. At the preoperative anesthesiology evaluation, vital signs were unremarkable. A medical abortion was performed, followed by Dilation and Curettage (D&C) under epidural anesthesia. The D&C lasted five minutes and immediately after the procedure, the patient developed a severe hypertensive crisis with cardiogenic shock. The patient required intensive resuscitation procedures, including inotropic agents and extracorporeal membrane oxygenation. She subsequently developed hepatic failure that led to coagulopathy. A computed tomography scan revealed a previously unknown left perirenal mass. Eventually, the patient died due to multiorgan failure. An autopsy was ordered to clarify the cause of death and to assess any medical responsibility. At the internal examination, the left mass was found to originate from the adrenal gland; upon gross examination, the cut surface of the mass was diffusely hemorrhagic. Histopathology of samples from the adrenal mass using hematoxylin-eosin stain was suggestive for PHEO.3 The immunohistochemistry analysis on the same samples demonstrated strong and diffuse positivity for chromogranin A in all fields. In addition, a medical inquiry with the woman’s relatives revealed that the deceased was affected with hypertension treated with triple-combination therapy. In the light of these findings, death was ascribed to an acute hypertensive crisis caused by an occult adrenal PHEO. PHEO has been referred to as the great mimic because the symptoms are quite common and sometimes it can be suspected only after paroxysmal episodes such as hypertensive crises. Unmanaged and/or undiagnosed PHEO is dangerous and can lead to serious and potentially lethal cardiovascular complications due to the acute massive secretion of catecholamines.4 In this case, the patient was affected by an undiagnosed PHEO that triggered a lethal hypertensive crisis during a minimally invasive gynecological procedure under local anesthesia. Medical malpractice was excluded because the tumor was occult and the patient did not report her complete medical history. In conclusion, an unknown PHEO can lead to sudden and unexpected death in young patients. In some instances, the diagnosis of PHEO can only be formulated after a complete medicolegal investigation. Reference(s): 1. Harari, A.; Inabnet, W.B. III. Malignant pheochromocytoma: A review. Am J Surg. 2011;201(5):700-708. 2. Bartikoski, S.R.; Reschke, D.J. Pheochromocytoma Crisis in the Emergency Department. Cureus. 2021;13(3):e13683. Published 2021 Mar. 3. Sherwin, R.P. Histopathology of pheochromocytoma. Cancer. 1959; 12:861-877. 4. Naranjo, J.; Dodd, S.; Martin, Y.N. Perioperative Management of Pheochromocytoma. J Cardiothorac Vasc Anesth. 2017;31(4):1427-1439.
2022
A responsive Academy - meeting and surpassing the challenges of a modern forensic science world
Seattle - WH
21-25 february 2022
Proceedings
AAFS
760
760
Sudden and Unexpected Death, Pheochromocytoma, Abortion
Lupariello, Francesco; Mirri, Federica; Innocenzi, Fabio; Gabriele, Alessandro; Di Vella, Giancarlo;
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2318/1849719
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