Critical Care: Student/Resident Case Report Poster - Critical Care II |

Far Reaching Consequences of Carcinoid Tumor of the Middle Ear: The First Reported Case of CTME With Dural Sinus Thrombosis and Distant Metastasis FREE TO VIEW

Biplab Saha, MD; Himani Sharma, MD; Kristin Fless, MD; Paul Yodice, MD; Fariborz Rezai, MD; Nirav Mistry, MD; Vagram Ovnanian, MD
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Saint Barnabas Medical Center, West Orange, NJ

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):408A. doi:10.1016/j.chest.2016.08.421
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SESSION TITLE: Student/Resident Case Report Poster - Critical Care II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Neuroendocrine tumor of middle ear is extremely rare. Over the past 40 years, many names have been ascribed to these seemingly benign neuroendocrine lesions including middle ear adenoma (MEA), adenomatous tumor of the middle ear and carcinoid tumor (CT). The term ‘Neuroendocrine adenoma’ has also been used to better describe the histologic nature of these tumors. Here we present the first case of Carcinoid tumor of middle ear (CTME) complicated by dural sinus thrombosis and diffuse bony metastasis.

CASE PRESENTATION: A 73 year old man presented with right sided lower motor neuron (LMN) type facial nerve palsy of 4 hour duration and ongoing progressive hearing loss for 2 months. Physical examination revealed inflamed and perforated tympanic membrane with purulent material draining from the right ear. Laboratory findings were unremarkable except an alkaline Phosphatase level of 816. A CT scan of the head showed a small right cerebellar infarct and opacification of the right mastoid air cells. The MRI demonstrated subacute right cerebellar hemorrhagic venous infarct. MRV revealed thrombus in the right sigmoid and transverse sinus. A heterogeneous middle ear mass extending into the internal auditory canal was seen. Cervical spine MRI revealed diffuse osseous metastases. He underwent surgical resection of the mass. The histology and immunohistochemistry (IHC) was consistent with CTME. The IHC was variably reactive for cytokeratin 20 (CK20), AE1/AE3, CAM5.2, OSCAR, synaptophysin, CD10 and TTF1. A proliferation rate of>75% was seen by Ki67 staining. He unfortunately suffered from massive intracranial bleed on heparin therapy and passed away.

DISCUSSION: The neuroendocrine tumors of head neck region are divided in 3 categories based on histology and IHC. 1) Well differentiated CT, <2 mitotic count/10HPFs, Ki-67<2% 2) Atypical carcinoid with 2-10mitotic count/10HPFs, Ki-67 between 3-20% and 3) Small cell cancer, mitotic count of >10/10 HPFs and Ki-67>20%. Although regional metastasis is not uncommon, only 2 cases of distant metastasis have been reported in the past. Our patient was diagnosed to have atypical carcinoid but the Ki-67 was >70% which is consistent with small cell cancer. Carcinoids are a known cause of hypercoagulable state and rarely cause dural sinus thrombosis. However, this is the first case of dural sinus thrombosis with CTME. Whether local invasion or metastatic disease or hypercoagulable state or all of them are responsible, is unknown.

CONCLUSIONS: Whether CTME and MEA represent the same entity is still debatable. The general term ‘Neuroendocrine adenoma’ probably doesn’t apply to all these tumors since they are known metastasize. More research is necessary to find out the biological markers for better prognostication of this rare cancer.

Reference #1: Kevin R. Torske, D.D.S., M.S., Lester D.R. Thompson, M.D. Adenoma versus Carcinoid Tumor of the Middle Ear: a Study of 48 Cases and Review of the Literature. VOL. 15, NO. 5, P. 543, 2002

DISCLOSURE: The following authors have nothing to disclose: Biplab Saha, Himani Sharma, Kristin Fless, Paul Yodice, Fariborz Rezai, Nirav Mistry, Vagram Ovnanian

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