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Open Access 01-10-2017 | Brief Report

Composite tumor with pheochromocytoma and immature neuroblastoma: report of two cases with cytogenetic analysis and discussion of current terminology

Authors: Lily Tran, Carrie Fitzpatrick, Susan L. Cohn, Peter Pytel

Published in: Virchows Archiv | Issue 4/2017

Excerpt

Pheochromocytoma, paraganglioma, and neuroblastoma are all derived from sympathetic nervous tissues and can exhibit a spectrum of morphology. The literature includes reports of tumors with morphologic features of both pheochromocytoma and neuroblastoma within the same tumor. The term “composite pheochromocytoma” has been used to describe these tumors as well as pheochromocytomas with other neural crest derivatives, such as malignant peripheral nerve sheath tumor and neuroendocrine carcinoma. “Composite pheochromocytoma” is rare, and there is a paucity of information in the literature regarding its biological behavior, clinical outcome, and molecular profile. The most commonly reported cases include components of pheochromocytoma and ganglioneuroma [1]. However, there are only few reports of tumors with components of pheochromocytoma and stroma-poor neuroblastoma (Table 1) [2‐7]. We report two distinct cases of composite tumors of the adrenal gland consisting of pheochromocytoma and neuroblastoma.

Table 1

Summary of previously reported cases of composite pheochromocytoma with immature neuroblastoma component including the two cases reported in this study

Case number	Reference	Age (years)/sex	Associated syndromes	Location	Metastasis	Treatment	N-MYC amplification	Follow-up
1	Wahl 1943 [2]	4/M	None	Mediastinum	Yes	Radiation	N/A	Died (8-month follow-up)
2	Franquemont 1994 [3]	49/M	None	Adrenal-left	No	Surgery	N/A	No evidence of disease after 1 year
3	Franquemont 1994 [3]	38/M	None	Adrenal-right	Yes	Surgery and chemoradiation	N/A	Died 3 months after diagnosis
4	Candanedo-Gonzalez 2001 [4]	56/F	None	Adrenal-right	No	Surgery	N/A	Asymptomatic at 24 months post-op
5	Tatekawa 2006 [5]	5/M	None	Adrenal-left	No	Surgery	N/A	Unknown
6	Comstock 2009 [6]	17/M	NF1	Adrenal-laterality unspecified	No	Unknown	Non-amplified	Alive (follow-up date unknown)
7	Steen 2014 [7]	46/M	None	Adrenal-left	No	Surgery	N/A	Asymptomatic and normotensive at 6 months post-op
8	Current study	57/M	NF1	Adrenal-left	No	Surgery	Non-amplified	Asymptomatic and normotensive (6 months post-op)
9	Current study	5/M	None	Adrenal-left	Yes	Surgery, chemoradiation, SCT	Amplified	Died 2 years after diagnosis

NF 1 neurofibromatosis type 1, SCT stem cell transplant

…

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Title: Composite tumor with pheochromocytoma and immature neuroblastoma: report of two cases with cytogenetic analysis and discussion of current terminology
Authors: Lily Tran
Carrie Fitzpatrick
Susan L. Cohn
Peter Pytel
Publication date: 01-10-2017
Publisher: Springer Berlin Heidelberg
Published in: Virchows Archiv / Issue 4/2017
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-017-2225-9

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Composite tumor with pheochromocytoma and immature neuroblastoma: report of two cases with cytogenetic analysis and discussion of current terminology

Excerpt

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