Thosar MA*, Shetty A**, Gupta AS***.
(* Assistant Professor, ** Second year resident, *** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College & KEM hospital, Mumbai, India.)
Primary non-Hodgkin lymphoma (NHL) rarely involves gynecological tract and is not considered in the differential diagnosis of bilateral ovarian masses. We present a case with bilateral ovarian masses who presented in emergency with intestinal perforation. On histopathology it was diagnosed as non-Hodgkin lymphoma.
NHL involving gynecological tract is very rare and is generally not considered in the differential diagnosis of bilateral ovarian tumors. Though rare, ovarian involvement is most often found amongst genital tract. Up to 35% of primary ovarian tumors are bilateral and are similar to Krukenberg tumor. Herewith we present a case of ovarian NHL that was diagnosed during laparotomy that was done for intestinal perforation. It supports the same Krukenberg concept of spread of NHL to ovary.
A 23 year old unmarried girl was referred to the emergency of our tertiary care hospital with complaints of pain in abdomen and recurrent vomiting since three days. She had no significant past medical or surgical history except appendicectomy four years back. She had no menstrual complaints, and her menstrual cycles were regular.
On presentation she had no pallor, pulse rate was 128/ minute, blood pressure was in the normal range and at presentation patient was afebrile. There were no palpable cervical or axillary lymph nodes. Central venous pressure (CVP) was 8 cm of water. On abdominal examination, she had guarding, rigidity as well as rebound tenderness. Bowel sounds were absent.
On investigation hemoglobin was 11.4 gm%, total leucocyte count was 14500/ ml; on differential counts neutrophilia was present. Liver and renal function serological tests were normal. The patient had a computed tomography (CT) scan done before referral. It was suggestive of thickening and loss of gastric folds, collapsed small bowel with multiple air fluid levels as well as free air in the abdomen. Many enlarged celiac, perigastric, portal, aorto-caval and mesenteric lymph nodes were seen. There was copious free fluid in the abdomen suggestive of perforative peritonitis. Imaging also showed bilateral ovarian masses right greater than the left. They were solid, and homogeneous. Tumor markers for ovarian malignancy were sent.
Figure 1. Radiograph of the abdomen in standing position showing gas under the diaphragm and multiple air fluid levels.
Figure 2. CT scan pelvis. A is right ovarian mass and B is left ovarian mass.
An emergent exploratory laparotomy was performed for perforative peritonitis. On opening the abdomen there was a transverse intestinal perforation of 2.5 cm about 150 cm proximal to the ileocecal junction (ICJ). There were multiple impending perforation sites over the ileal segment of 10-12 cm. The base of the perforation was necrotic. Adjacent omentum was thickened. Celiac, perigastric and mesenteric lymph nodes were enlarged. Bowel segment was resected and a double barrel ileostomy was performed by the surgeons.
Figure 3. Resected segment of ileum. (A is the intestinal perforation. B, C and D are impending perforation sites).
Figure 4. Bilateral ovarian masses. (A is deposit of tumor on the left ovary. B is right ovary replaced by tumor. C is the uterus).
There were bilateral ovarian masses, red fleshy, firm in consistency with an irregular surface. Right ovary was completely replaced with mass of 6×7 cm along with capsular breach. There was a deposit of similar mass of 3×3 cm on the surface of the left ovary. Right side salpingo-oophorectomy was done and wedge resection of the left ovary was performed.
She developed massive intra abdominal hemorrhage on day 2 requiring re-exploration to control the bleeding. The tumor marker report that came subsequently showed elevated Lactate dehydrogenase (LDH) (1231 U/lit) levels. She developed disseminated intravascular coagulopathy and sepsis and succumbed on day 7 despite multiple transfusions and replacement of clotting factors. The relatives did not consent for an autopsy.
Final histopathology report showed the right ovary with features of non-Hodgkin lymphoma, wedge resection of other side ovary was normal. Bowel had ischemic necrosis and perforation, though no malignancy was identified.
Lymphoma rarely occurs in the female genital tract. The incidence of primary lymphomas in the genital tract is reported to be 0.2 to 1.1% of the total lymphomas.[2,3] Secondary involvement is more common than primary and is estimated to be 7% of total lymphoma cases. In only up to 35% of cases, primary ovarian lymphoma is bilateral while bilateral involvement was more often in secondary ovarian lymphomas. Involvement of the uterus and ovaries by lymphoma is usually found at the time of autopsy performed after death due to advanced disease, suggesting metastasis of the disease to these organs similar to the Krukenberg concept of spread.
Though rare, the ovary is the most common organ in the gynecological tract to be involved by NHL. Malignant lymphoid precursors may present as a primary ovarian neoplasm or as a secondary dissemination from a distant disease. The median age of presentation in primary malignant ovarian lymphomas is 45 years while for secondary malignant ovarian lymphomas median age of presentation is 33 years.[1,5] Only Burkitt’s lymphoma presents at an early age (median age of 20 years).
Presentation of primary or secondary ovarian lymphoma can be variable, with accidental discovery on radiological scanning, to overt genital bleeding or abdominal symptoms like swelling, distension and pain. Liang et al. suggested a strong correlation of gastrointestinal tract with ovarian lymphoma similar to our case report. Christopoulos reported a case with NHL involving stomach and ovary and found a similar correlation. He also proposed to extend Krukenberg concept to include ovarian NHL.
In 33 years single institute experience of genital NHL, Ahmad et al. found radiological imaging modalities were unable to differentiate between lymphoma from other malignancies. Primary ovarian lymphoma was found to distort the internal structure of the ovary and often presented with a large ovarian mass.
To classify NHL involving genital tract the World Health Organization classification is used which depends on incisional or excisional biopsies for diagnosis. Fine needle aspiration biopsies fail to demonstrate required cell-to-cell architecture for accurate diagnosis. Diffuse large B-cell NHL is the most common type of NHL involving the female genital tract. Though primary gonadal disease evaluation reveals more advanced spread (FIGO stage 2–4), Vang et al. have reported better treatment outcome compared to secondary disease. With systemic chemotherapy, up to 75% patients with localized disease were found to have better long-term survival and hence is recommended. The survival in ovarian NHL and extra nodal NHL is similar with chemotherapy. In cases of secondary involvement of the ovary, survival depends on the type of the lymphoma as well as other prognostic indices like age, tumor stage, the number of involved sites, performance status, and lactate dehydrogenase (LDH) levels. Due to the rarity of the condition no standard treatment protocols exist, still surgical cytoreduction followed by combination chemotherapy is used most commonly.
In our case due to multiple lymph node involvement in the celiac axis and small bowel ischemic ulcers, we believe ovarian mass was secondary to an undiagnosed primary in the stomach or the small bowel similar to a Krukenberg tumor. We postulate that metastatic tumor cells obliterated the mesenteric blood vessels leading to ischemic necrosis and small bowel perforations as the resected segment of the ileum was negative for malignancy. Unfortunately, we could not locate the primary as relatives did not consent for a pathological autopsy. Had the primary been diagnosed it would have lent support to the Krukenberg concept of spread to include ovarian NHL. With this case report we want to emphasize that while considering differential diagnosis of bilateral ovarian masses one should be vigilant, and even rare causes like NHL should be considered. To achieve good outcomes multimodality treatment should be planned for these patients.
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Thosar MA, Shetty A, Gupta AS. Krukenberg tumor: Non Hodgkin Lymphoma. JPGO 2016. Volume 3 No. 11. Available from: http://www.jpgo.org/2016/11/krukenberg-tumor-non-hodgkin-lymphoma.html