Paraneoplastic pemphigus as a first sign of metastatic retroperitoneal inflammatory myofibroblastic tumor: (18)F-FDG PET/CT findings

Rev Esp Med Nucl Imagen Mol. 2016 Jul-Aug;35(4):260-2. doi: 10.1016/j.remn.2015.09.005. Epub 2015 Dec 28.
[Article in English, Spanish]

Abstract

A 30-year-old female presented with a 3-month history of erosive stomatitis and bullous lesions, along with recurrent episodes of abdominal pain. She was found to have a retroperitoneal lump in left lumbar region. Skin biopsy revealed bullous disorder. CT guided biopsy of the retroperitoneal mass was suggestive of inflammatory myofibroblastic tumor (IMT). She was started on oral steroids and supportive care, and surgery was being planned when she developed respiratory failure. CT chest revealed vertebral metastases. PET/CT for whole body work up revealed a left para-aortic mass along with multiple skeletal metastases. The patient was kept on conservative management. After 3 months, the patient has shown clinical improvement, and an exploratory laparotomy is now being planned for the excision of the tumor, followed by chemotherapy. This case of retroperitoneal IMT is rare in terms of skeletal metastases with paraneoplastic pemphigus.

Keywords: (18)F-FDG PET/CT; (18)F-FDG PET/TC; Inflammatory myofibroblastic tumor; Metastatic; Metastásico; Paraneoplastic pemphigus; Pénfigo paraneoplásico; Retroperitoneal; Tumor miofibroblástico inflamatorio.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Fluorodeoxyglucose F18*
  • Granuloma, Plasma Cell / diagnostic imaging*
  • Humans
  • Paraneoplastic Syndromes / diagnostic imaging*
  • Pemphigus / diagnostic imaging*
  • Positron Emission Tomography Computed Tomography*
  • Radiopharmaceuticals*
  • Retroperitoneal Neoplasms / diagnostic imaging*
  • Retroperitoneal Neoplasms / secondary*

Substances

  • Radiopharmaceuticals
  • Fluorodeoxyglucose F18