Benign metastasic leiomyomatosis: A rare case with bone affection

Clinical Cases 17.03.2023
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 50 years, female
Authors: María Elena Orihuela de la Cal, Carlos Alberto Machado Orihuela
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Details
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AI Report

Clinical History

A 50-year-old female was admitted for management and conduct of incidental cervical mass with suspected diagnosis of nerve sheath tumour. Surgery was performed, with histologic results of leiomyomatosis lineage. Studies before surgery were performed in another centre.

Imaging Findings

Post-surgical cervical CT confirmed a residual mass at the level of C3 to C5 and blastic lesions.

Body CT revealed the presence of small isolated lung nodules. Solid masses in the peritoneal cavity and in the abdominal wall, with heterogeneous attenuation. Many blastic lesions were seen in the vertebral bodies, ribs and pelvis, suggested of metastasis as the first diagnosis.

Spine MR showed many lesions with contrast enhancement, some of them with soft tissue component that affected the right foramen of D5 and D12.

Bone scintigraphy showed no “hot spots” suggestive of metastasis, considering the study results to be indeterminate.

A biopsy of the abdominal wall mass was performed with histological results compatible with leiomyoma, with positive estrogen and progesterone receptors.

Bone biopsy of the pelvis lesion was also performed, with histological results of focal infiltration by mesenchymal neoplasia with an immunoprotib compatible with leiomyomatous lineage.

Discussion

Benign metastasic leiomyomatosis (BML), is a rare entity, characterized by proliferation of smooth muscle fibre in remote places from the uterus. Bone affection is very rare, being the lung the most frequent place of appearance. Frequently presents with a radiological appearance similar to metastasic disease. The basic pathogenesis is a multicentric metaplasic change of the mesothelial connective tissue, due to an abnormal response to ovarian hormonal simulation.

Most cases are asymptomatic and are discovered incidentally in image studies. History of hysterectomy for uterine leiomyomas can guide the diagnosis.

Ultrasound, CT and MR imaging features are useful to identify and stage the disease. It can be presented as solid and complex soft tissue masses in the peritoneum cavity, with heterogeneous enhancement. It´s not associated with infiltration of omentum, ascites or liver metástasis. The diagnosis can be confirmed by biopsy, high levels of positive estrogen and progesterone receptors can also be found.

Recurrence is possible and with each one, is more likely to produce sarcomatous degeneration, although is uncommon. Prognosis is good in postmenopausal patients, although it is variable. Cases of progression have been described and are usually related to high levels of estrogen.

Written informed patient consent for publication has been obtained.

Differential Diagnosis List

Benign Metastasic Leiomyomatosis
Malignant tumour metastasis

Final Diagnosis

Benign Metastasic Leiomyomatosis

Figures

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Axial cervical spine CT showing a mass in right foramen of C3-C5 level (arrow)
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Sagittal cervical spine CT reconstruction with bone filter showing post-surgical changes and blastic lesion in D2 body (arrow

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Axial lung CT showing small lung nodules (arrows)
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Axial abdominal CT showing an intra-abdominal heterogeneous mass (arrow) with enhancement in the right side of the pelvis
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Axial abdominal CT showing a nodule in the abdominal wall (arrow) with heterogeneous enhancement
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US of the same lesion, showing hypoechoic pattern with internal small anechoic areas
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Axial head CT showing a soft tissue nodule (arrow) with enhancement
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Sagittal lumbar spine CT with bone filter reconstruction showing multiple blastic lesions in vertebral bodies
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Axial pelvic CT with bone filter showing multiple blastic lesions in both iliac bones

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Axial T2w cervical spine MR showing a lesion (arrow) with intermediate signal in the right foramen of C3-C5
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Sagittal T1 FAT-SAT lumbar spine MR without contrast showed multiple low and intermediate signal lesions in the vertebral bod
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Sagittal T1 FAT-SAT lumbar spine MR with contrast showed heterogeneous enhancement of the lesions. The pelvic mass is partial

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Bone scintigraphy showing no “hot spots” suggestive of metastasis