Diffuse peritoneal leiomyomatosis

Case contributed by Ashesh Ishwarlal Ranchod
Diagnosis certain

Presentation

Non-specific abdominal pain, for workup. No other significant symptoms and the confirmed absence of weight loss.

Patient Data

Age: 50 years
Gender: Female
ultrasound

There are multiple intraabdominal and intrapelvic large, solid mass lesions. The larger lesions measure: central pelvis 12.4 x 10.1 x 9.8 cm, infra umbilical 7.0 x 6.6 x 7.23 cm, and right para-umbilical 9.0 x 8.0 x 9.2 cm. There is no abnormal hyperemia or vascularity and no associated calcifications are identified.

Sonographically, there is no hydronephrosis or hydroureter to suggest extrinsic urinary tract obstruction. The bladder is non-distended and grossly normal. There is a known hysterectomy.

ultrasound

Cine loops demonstrate the solid mass lesions within the abdomen and pelvis. They appear as circumscribed and discrete masses with no calcification.

There are multiple, predominantly central, solid, lobulated, circumscribed, intraabdominal and intrapelvic enhancing masses. There is a conglomerate, central to right-sided, eccentric mass measuring 15.67 x 10.93 x 7.57 cm. This displaces regional bowel loops peripherally. There is an extrinsic mass effect on the adjacent displaced right ureter with upstream hydroureter and hydronephrosis.

There is a left pararenal mass measuring 5.5 x 3.9 x 5.74 cm with mild extrinsic mass effect on the left ureter and consequent hydroureter and hydronephrosis.

There is a right adnexal mass measuring 11 x 8.65 x 3.21 cm with central and eccentric necrosis. This displaces the bladder anteriorly and towards the left. There is a left adnexal mass measuring 7.8 x 7.31 x 8.42 cm displacing the bladder anteriorly. This latter lesion is inseparable from the vaginal vault.

The uterus is absent. No significant or suspicious paraaortic or peritoneal lymphadenopathy is present. There is no ascites. There is no bowel obstruction and no pulmonary parenchymal, hepatic , or osseous metastases. Suspected lipid poor,non calcified, no enhancing pulmonary hamartoma within the left lower lobe.

There are vulvar/ perivulvar varices which additionally extend up the right lateral abdomen

Case assistance : Dr Pearl GNM Quvane.

pathology

There are two specimens weighing a total of 779.50 g. The larger lesion measured 155 x 85 x 75 mm and the smaller lesion measured 80 x 80 x 60 mm. Histological features are consistent with leiomyoma with smooth muscle bundles arranged in short intersecting fascicles. The cells have bright eosinophilic cytoplasm with cigar-shaped nuclei and no atypia. There are no mitotic figures and no stromal atypia. There is no coagulative necrosis.

Case Discussion

A histopathologically proven case of disseminated peritoneal leiomyomatosis in an adult patient with a confirmed history of hysterectomy approximately 15 years ago. The hysterectomy was an open surgical procedure rather than a laparoscopic one and the likely use of a power morcellator is not clinically suspected. Based on the sonographic and CT appearance, offered differentials included lymphoma, metastatic lymphadenopathy of an occult/ unknown primary malignancy and diffuse peritoneal carcinomatosis.

Surgery was planned as a 2 step procedure, to initially excise some lesions and obtain excision histology and return for a more definitive combined surgical and gynecological procedure at a delayed stage based on histological results.

The CT was performed as a staging study from the thorax to the symphysis pubis and image acquisition is significantly degraded due to the elevated BMI and body habitus of the patient as appreciated by the CT scout views and grainy venous phase.

The lobulated left lower lobe mass lesion is non-enhancing and the differentials include a hamartoma. Benign metastasizing leiomyoma is a rare possibility in this instance and can only be proven on histology.

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