We report a case of a 68 years old woman presenting to the emergency department with macrohematuria and dysuria. She experienced fever several times, starting two months before medical examination. Fever was treated with NSAID. A history of recent pelvic pain was also reported. Her clinical status was good and no other symptoms or concurrent illnesses were present at the time of hospitalization.
She was admitted to Gynecologic service for further evaluation.
The patient was virgin and in post-menopausal status. Clinical history was unremarkable; negative either for previous medical or surgical procedures. Family history was positive for cancer with one male sibling deceased from a pancreatic malignancy; the remainder two siblings had no positive history for malignancies as well as parents and closest relatives. Social history was negative for use or addiction to drugs, alcohol and exposure to potential risk factors.
A digital rectal examination had to be performed in place of the gynecologic bimanual examination. A gross, firm mass located in the pelvis was found at physical examination.
Pelvic US, abdominal CT scans, descending pielography, cystoscopy and chest RX were performed to both assess local disease and evaluate the presence of local and distant metastases.
Computed tomography imaging confirmed the presence of a complex mass in the pelvis (13 × 14 cm) showing irregular contours and small areas of calcifications. Uterus and adnexa could not be clearly recognized, showing as being part of the mass. Iliac vessels and bladder were displaced. Imaging technique reports stated that no vessels or bladder invasion was present. A hypoplasia of the left kidney was also reported. No other metastasis was reported in the abdomino-pelvic cavity.
Pielography showed a delay in the opacification of the renal pelvis associated to a dilation of the right ureter that was compressed and displaced in its pelvic course till entering the bladder. No opacification of the contra lateral kidney and excretory ways could be obtained.
Cystoscopy showed, eventually, a bleeding lesion onto the bladder wall that raised suspicion of neoplastic invasion. The lesion appeared more likely as a loss of continuity of the urothelial mucosa; an ulcerating mass was visualized and eventually described on final report.
Chest RX and bone scan were negative for distant metastases.
A multidisciplinary board, including a urologist, a radiologist, a radiation oncologist, an oncologist and a gynecologist, evaluated the collected data. It was eventually decided to surgically extirpate the tumor. A transurethral biopsy of the vesical wall lesion was not considered since major suspicion was addressing to uterine or adnexal neoplasms and the high stage was reasonably an indication to undergo definitive surgery.
Surgery was eventually performed after pre-operative routine and evaluation.
A longitudinal abdominal incision was preferred to access peritoneal cavity. A bulky mass was found in the pelvis. Uterus and adnexa appeared macroscopically free from neoplastic invasion. The mass showed a cleavage with recto-sigmoid tract, iliac vessels and pubic bone, whether uterus, adnexa, right ureter and left obturator nerve could not be separated from the tumor. A histological intra-operative examination revealed a malignant, spindled cell neoplasm. Surgery had to include a wide mass excision, in order to obtain free margins and control of local disease. Final treatment choice was a pelvic exenteration. This decision was made since local disease was advanced and it was set to obtain specimen's margins disease-free as a primary target.
An en-bloc resection of the mass, together with uterus, adnexa, bladder, distal part of right ureter and left obturator nerve was performed (anterior pelvic exenterantion).
A cutaneous ureterostomy was placed in the site of ureteral dissection. The decision to perform this procedure in place of a standard urinary diversion, as a ileal conduit or Miami pouch, was made on the evidence of poor prognosis that appeared from surgical exploration and intra-operative histology that was addressing to a malignant stromal neoplasm. It seemed to be reasonable to lower intra- and post-operative morbidity whereas the potential overall survival of the patient appeared to be prospectively low.
A final histological diagnosis of high-grade leiomyosarcoma, (Fig. ), (G3, FNCLCC 1986) pT2b pN0 pM0, AJCC (2002) Stage III, was made based on immunoreactivity to smooth muscle actin, score 2 (Fig. ). The specimen resulted negative to c-kit and EGFR. Proliferation index was 90%, evaluated thru MIB-1 (Ki-67) (Fig. ). Necrosis was inferior to 50% (score 2) and mitotic index higher than 20 mitosis per field/10 HPF (score 3). The tumor presented, macroscopically, as a bulky, brain-like, white-grayish mass. The tumor contained diverse necrotic and hemorrhagic areas, and invaded the vesical wall. The size of the mass was 14 × 11 × 6.5 cm. Histology confirmed that uterus and adnexa were free from neoplastic invasion. Surgical margins resulted negative at the final examination. LVI was not reported.
Leiomyosarcoma of the bladder (Hematoxylin and eosin).
Leiomyosarcoma of the bladder (Smooth muscle actin).
Leiomyosarcoma of the bladder (Ki67).
Post-operative course was free from complications and the patient could be discharged from the hospital fourteen days after surgery.
The patient underwent chemotherapy one month after surgery, using doxorubicin as a single agent in an adjuvant setting. She died after the first cycle from a distant recurrence to the left lung.