A 55 year old postmenopausal woman presented with complaints of sudden onset of right sided abdominal pain restricting her daily activities. Physical examination showed a large, smooth and tender immovable mass, extending from right lumbar region to the right anterior superior iliac spine and extending beyond the midline of the abdomen. On per speculum examination the cervix could not be visualised and to the extent vagina was seen, appeared normal. On per vaginal and rectal examination, cervix was pulled up. Uterus felt atrophic and retroverted, both the fornices were free. On the left in a plane above the adnexa, a firm mass could be tipped on downward displacement of the abdominal mass. On per rectal examination, rectal mucosa was free, bilateral parametrium were supple, pouch of Douglas was free and the same mass could be tipped with the middle finger. Complete haemogram and biochemical investigations were within normal limts.CA-125-was 8 IU/L. A CT scan of abdomen and pelvis was done with contrast. It showed a large retroperitoneal mixed attenuation mass 18.7×12.7×15.5 cms with density value of +20-30Hounsfeild units (Fig. 1).During surgery, minimal haemorrhagic ascites was seen in the peritoneal cavity. A well encapsulated retroperitoneal mass between the leaves of the broad ligament was completely resected, clamping its attachments to the parametrial and lateral pelvic wall and other soft tissues (Fig. 2). Frozen section was reported as lipomatous tumour. Total hysterectomy with bilateral salphingo-oophorectomy was then performed.
PathologyGrossly the tumour was irregular, whitish yellowish, bosselated with surface vessels and measuring 30×23.5×25 cms. Cut section showed lobules of fatty tissue, white fibrous and myxoid areas (Fig. 3). Uterus and bilateral tubes and ovaries were normal grossly and histologically.Histology of the tumor consisted of interlacing bundles of spindle cells and foci of thick and thin walled blood vessels in a background of mature adipocytes suggestive of angiomyolipoma (Fig. 4). Interestingly immunohistochemistry with antihuman melanoma HMB-45 was negative.Post operative period was uneventful. Patient is on follow up for the last 13 months and does not have a recurrence clinically or sonographically.
DiscussionThe incidence of renal and extra renal angiomyolipoma is 0.3-3 % [1] and that of angiomyolipoma occurring between the leaves of the broad ligament is very rare, only one case by Rajan and et al has been published [2]. Probably ours is the second case reported till date. Acute pain could be due to ischemia of the tumour mass or an impending rupture of the vessel. On reviewing the literature extra renal angiomyolipoma occurring in the female genital tract has been reported