Contributed by John A Ozolek, MD and Jean C Dunn, MD
Published on line in April 2002
This is a 64-year-old woman initially diagnosed with acute myelogenous leukemia (FAB-M1) She underwent one cycle of Ara-C and Mitomycin with no response. She underwent eight cycles of Interleukin 2. This was followed by a recurrence and she underwent a non-myeloablative allogeneic stem cell transplant with myeloablative chemoradiation followed by Fludarabine for three days and low-dose total body radiation. She received the donor bone marrow from a 6/6 HLA identical matched male sibling. She was currently 21 months status post her stem cell transplantation. She has had in the past graft versus host disease of the small bowel and also graft versus host disease of the liver treated with high-dose steroids and hyperalimentation. She had a complete remission of her leukemia following the allogeneic transplant. She complained of mild abdominal bloating and discomfort beginning three months prior to her death. She stated that at that time, she was nauseated with some emesis. She also had noticed increased abdominal gas and bloating and mild early satiety. She received minimal relief from over the counter medications for these symptoms. Her symptoms continued to worsen and she was seen for routine follow-up one month later. At that time, she was complaining of persistence of abdominal cramping but no fever, chills, night sweats or persistent nausea or vomiting. She did state she was unable to lie down flat, but denied any changes in her stool habits, but did note increased urinary frequency. At that time, she was given a prescription for Nexium and no further workup was performed. Since the three weeks following that visit, she noted an increase in her abdominal swelling as well as her abdominal complaints. She saw her primary care physician the week prior to her admission who performed an abdominal x-ray, which revealed distended loops of bowel suggestive of a possible obstruction. She then began to have significant left and right lower quadrant pain with persistence of bloating and mild early satiety and she was admitted for further workup. On her initial exam, upon admission, she was noted to have a protuberant abdomen with a large pelvic mass extending approximately 12 cm cephalad from the pubic symphysis that was firm but non-mobile. There was also a large mass in the right upper quadrant which measured 10 x 8 cm which was firm and had irregular margins and minimally mobile. She had diffuse nodularity throughout the abdomen extending into the left side and left upper quadrant. There was no hepatosplenomegaly. She underwent an exploratory laparotomy secondary to bowel obstruction. During the procedure an omental, peritoneal, and uterine biopsy were performed. She was begun on Hydroxyurea and monitored closely for tumor lysis syndrome. Her white blood cell count on 2/23/02 was 11.1 with 75% neutrophils, 7% lymphocytes, 11% band forms, 3% monocytes, 2% blasts, and 1% metamyelocytes. Her hemoglobin was 10.5 and platelet count 234 thousand. She was found unresponsive by her nurse. Despite resuscitative measures, she died.
Fig. 1: This image shows extensive mesenteric and retroperitoneal lymphadenopathy with dilated loops of small bowel.
Fig. 2: This image shows a mass in the inferior aspect of the abdomen extending into the pelvis. This mass was interpreted to be indistinguishable from the uterus or perhaps adjacent to the uterus.
Fig. 3: This image represents the extensive retroperitoneal lymphadenopathy seen on the computed tomography images. The tumor forms a 19.0 x 18.0 x 10.0-cm aggregate mass within the abdomen, which includes mesentery, omentum and encasing loops of small intestine. A 13.0 x 8.0-cm tumor also encompasses the aorta extending from the celiac axis to the bifurcations of the aorta (shown here).
Figs 4, 5: These images are taken of the pelvic mass previously described on computed tomography images. These are coronal cuts (more distant and close-up) of the markedly enlarged uterus (18.5 x 14.5 x 10.0 cm). The endometrium is thinned and red-brown. The endometrial cavity is triangular in shape and is 5.8 x 7.0 cm. The myometrium is firm and brown with a focal, 4.0-cm nodule of whorled brown tissue. The uterine wall is 6.0 cm thick and boggy with light pink parenchyma.