Open AccessComposite phaeochromocytoma with malignant peripheral nerve sheath tumour: A case report with summary of prior published cases
Author(s) -
Saswati Mukherjee,
Arnab Mondal,
Moumita Sengupta,
Uttara Chatterjee,
Debansu Sarkar,
Satinath Mukhopadhyay
Publication year - 2021
Publication title -
indian journal of pathology and microbiology/indian journal of pathology and microbiology
Language(s) - English
Resource type - Journals
eISSN - 0974-5130
pISSN - 0377-4929
DOI - 10.4103/ijpm.ijpm_524_20
Subject(s) - ganglioneuroblastoma , ganglioneuroma , neurofibromatosis , medicine , neural crest , neuroblastoma , pathology , pathological , immunohistochemistry , medullary cavity , biology , embryo , genetics , microbiology and biotechnology , cell culture
Composite phaeochromocytomas (CP) are extremely uncommon adrenal medullary tumours where phaeochromocytoma coexists with another adrenal medullary tumour also of neural crest origin. CP includes combination of phaeochromocytoma along with a component of neuroblastoma, ganglioneuroblastoma, ganglioneuroma, benign nerve sheath tumour or a malignant peripheral nerve sheath tumour (MPNST). Here we describe the morphological and immunohistochemical details of a case of CP with MPNST in a 30 years old lady, without history of neurofibromatosis. Only 6 cases of CP with MPNST have been reported so far. We have tabulated a summary of these prior published cases of phaeochromocytoma with MPNST. To our knowledge, this is the first literature review describing the clinico-pathological characteristics of these rare tumours.