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Inicio Revista Española de Medicina Nuclear e Imagen Molecular (English Edition) Paraneoplastic pemphigus as a first sign of metastatic retroperitoneal inflammat...
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Vol. 35. Issue 4.
Pages 260-262 (July - August 2016)
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Vol. 35. Issue 4.
Pages 260-262 (July - August 2016)
Clinical note
DOI: 10.1016/j.remnie.2015.09.007
Paraneoplastic pemphigus as a first sign of metastatic retroperitoneal inflammatory myofibroblastic tumor: 18F-FDG PET/CT findings
Pénfigo paraneoplásico como un primer signo de tumor retroperitoneal metastásico miofibroblástico inflamatorio: hallazgos 18F-FDG PET/TC
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V.S. Dhulla, A. Passaha, N. Ranab, S. Aroraa, S. Mallickc, R. Kumara,
Corresponding author
rkphulia@yahoo.com

Corresponding author.
a Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India
b Department of Radiodiagnosis, M S Ramaiah Medical College, Bengaluru, India
c Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
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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:
Inflammatory myofibroblastic tumor
Retroperitoneal
Paraneoplastic pemphigus
Metastatic
18F-FDG PET/CT
Resumen

Presentamos una mujer de 30 años de edad con una historia de 3 meses de estomatitis erosiva y lesiones ampullosas, junto con episodios recurrentes de dolor abdominal. La paciente había notado una tumoración en la región lumbar izquierda. La biopsia de piel reveló una enfermedad bullosa. La biopsia guiada por TC de la masa retroperitoneal fue sugestiva de tumor miofibroblástico inflamatorio (IMT). Se instauró tratamiento con esteroides orales y terapia sintomática. Durante la planificación de la cirugía desarrolló un cuadro de insuficiencia respiratoria. La TC de tórax reveló metástasis vertebrales. La PET/TC de cuerpo completo identificó una masa paraaórtica izquierda y múltiples metástasis esqueléticas. La paciente se mantuvo en tratamiento conservador. Después de 3 meses, la paciente ha mostrado mejoría clínica y, en la actualidad, está en periodo preoperatorio para laparotomía exploradora y extirpación del tumor seguida por quimioterapia. Este caso de IMT retroperitoneal es raro en términos de metástasis óseas con pénfigo paraneoplásico.

Palabras clave:
Tumor miofibroblástico inflamatorio
Retroperitoneal
Pénfigo paraneoplásico
Metastásico
18F-FDG PET/TC

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