Leiomyomatosis peritonealis disseminata: a rare disease with a difficult diagnosis

• Published in: BMJ case reports Vol. 12; no. 7; p. e229564
• Main Authors: Martins Jordão, Daniel, Santos Pereira, João, Furtado, Emanuel, Ferrão, Isabel Cristina
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 01-07-2019; BMJ Publishing Group
• Series: Case Report
• Subjects: Birth control; Case reports; Chemotherapy; Diagnosis, Differential; Endometriosis; Female; Fibroids; Gynecological cancer; Histopathology; Humans; Kidney - diagnostic imaging; Kidney Neoplasms - pathology; Kidney Neoplasms - surgery; Leiomyomatosis - pathology; Leiomyomatosis - surgery; Medical diagnosis; Medical imaging; Mesoderm - pathology; Metastasis; Middle Aged; Muscle, Smooth - pathology; Nephrectomy - methods; Patients; Peritoneal Neoplasms - diagnostic imaging; Peritoneal Neoplasms - pathology; Peritoneal Neoplasms - surgery; Rare Disease; Rare diseases; Smooth muscle; Treatment Outcome; Tumors; carcinogenesis; general surgery; gynecological cancer
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2019-229564

Leiomyomatosis peritonealis disseminata (LPD) is a rare condition, characterised by the proliferation of peritoneal smooth muscle nodules. LPD is a benign disease with a low rate of malignant degeneration. We describe the case of a 46-year-old, asymptomatic, woman presenting with a mass on the left renal hilum, identified by ultrasound. A CT scan showed three nodules near the left kidney, a mass anterior to the vena cava and bilateral iliac nodules. Biopsy revealed a mesenchymal low-grade tumour. The patient underwent a left nephrectomy and excision of the other masses. The histological diagnosis revealed smooth muscle nodular proliferation with no malignant features, compatible with LPD. The differential diagnosis between LPD and metastatic leiomyosarcoma is sometimes very difficult because they are clinically very similar and even on histology the diagnosis can be tricky. Treatment is conservative in most cases, with surgical excision reserved for high-risk patients.