Combined paraganglioma and IgG4-related retroperitoneal fibrosis

TL;DR

This paper reports a rare case of a 47-year-old woman with both a paraganglioma and IgG4-related retroperitoneal fibrosis, highlighting the challenges of managing these co-occurring conditions.

Contribution

The novelty lies in documenting a rare coexistence of paraganglioma and IgG4-related retroperitoneal fibrosis in a single patient.

Findings

The patient had a paraganglioma, a neuroendocrine tumor that can cause hypertension and sweating.

She also had IgG4-related retroperitoneal fibrosis, an inflammatory condition affecting the retroperitoneum.

The combination of these two conditions led to significant clinical challenges.

Abstract

A paraganglioma is a neuroendocrine tumor that may secrete catecholamines and present with symptoms of sympathetic overload such as hypertension and diaphoresis. It is important that paragangliomas are identified, as they must often be treated by surgical excision. IgG4-related retroperitoneal fibrosis (IgG4-RPF) is a systemic inflammatory disease that results in the infiltration of IgG4-positive plasma cells in the retroperitoneum. Such fibrosis may adversely affect nearby organs and tissues. Here, we describe a case of combined paraganglioma and IgG4-RPF in a 47-year-old female patient. This case demonstrates the deleterious effect of these two conditions when they occur simultaneously.

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