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Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature

BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid tissue while paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. Pancreatic FDCS associated with PNP and myasthenia gravis (MG...

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Autores principales: Lu, Tao, Song, Bin, Pu, Hong, Li, Xinglan, Chen, Qiqi, Yang, Chong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6570917/
https://www.ncbi.nlm.nih.gov/pubmed/31200650
http://dx.doi.org/10.1186/s12876-019-1008-y
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author Lu, Tao
Song, Bin
Pu, Hong
Li, Xinglan
Chen, Qiqi
Yang, Chong
author_facet Lu, Tao
Song, Bin
Pu, Hong
Li, Xinglan
Chen, Qiqi
Yang, Chong
author_sort Lu, Tao
collection PubMed
description BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid tissue while paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. Pancreatic FDCS associated with PNP and myasthenia gravis (MG) is even rarer and highly malignant. We present the clinical data, pathological materials and computed tomography (CT) features of a rare case of this disease. CASE PRESENTATION: A 49-year-old woman presented with repeated ptosis of both eyelids, oral ulcers and erosions. Her laboratory results showed a slight elevation of CA125 and positivity of some autoimmune antibodies. CT revealed a round solid mass with central necrosis in the pancreatic tail. The solid component of the mass showed slight enhancement and serpentine feeding arteries in the arterial phase, moderate enhancement with a draining vein around the tumor in the portal venous phase and persistent enhancement in the delayed phase. Surgical resection was performed, and the pathological diagnosis was FDCS. However, the patient died of inability to excrete sputum and occlusion of the respiratory tract. CONCLUSIONS: Pancreatic FDCS manifested as PNP and MG is very rare. Its CT features are not specific, and the disease should be differentiated from neuroendocrine tumors, solid pseudopapillary neoplasms and acinar cell carcinoma.
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spelling pubmed-65709172019-06-20 Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature Lu, Tao Song, Bin Pu, Hong Li, Xinglan Chen, Qiqi Yang, Chong BMC Gastroenterol Case Report BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid tissue while paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. Pancreatic FDCS associated with PNP and myasthenia gravis (MG) is even rarer and highly malignant. We present the clinical data, pathological materials and computed tomography (CT) features of a rare case of this disease. CASE PRESENTATION: A 49-year-old woman presented with repeated ptosis of both eyelids, oral ulcers and erosions. Her laboratory results showed a slight elevation of CA125 and positivity of some autoimmune antibodies. CT revealed a round solid mass with central necrosis in the pancreatic tail. The solid component of the mass showed slight enhancement and serpentine feeding arteries in the arterial phase, moderate enhancement with a draining vein around the tumor in the portal venous phase and persistent enhancement in the delayed phase. Surgical resection was performed, and the pathological diagnosis was FDCS. However, the patient died of inability to excrete sputum and occlusion of the respiratory tract. CONCLUSIONS: Pancreatic FDCS manifested as PNP and MG is very rare. Its CT features are not specific, and the disease should be differentiated from neuroendocrine tumors, solid pseudopapillary neoplasms and acinar cell carcinoma. BioMed Central 2019-06-14 /pmc/articles/PMC6570917/ /pubmed/31200650 http://dx.doi.org/10.1186/s12876-019-1008-y Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Lu, Tao
Song, Bin
Pu, Hong
Li, Xinglan
Chen, Qiqi
Yang, Chong
Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature
title Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature
title_full Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature
title_fullStr Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature
title_full_unstemmed Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature
title_short Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature
title_sort paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: ct findings and review of literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6570917/
https://www.ncbi.nlm.nih.gov/pubmed/31200650
http://dx.doi.org/10.1186/s12876-019-1008-y
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