RESUMEN
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 (AU)
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 (AU)
Asunto(s)
Humanos , Femenino , Adulto , Pénfigo/patología , Pénfigo , Neoplasias Retroperitoneales/complicaciones , Neoplasias Retroperitoneales/patología , Neoplasias Retroperitoneales , Metástasis de la Neoplasia/patología , Metástasis de la Neoplasia , Fluorodesoxiglucosa F18/análisis , Espacio Retroperitoneal/patología , Espacio Retroperitoneal , Tomografía de Emisión de Positrones/métodos , Tomografía de Emisión de Positrones/tendencias , Biopsia Guiada por Imagen , Laparotomía/métodosRESUMEN
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.
