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Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 663

High-grade non-Hodgkin's lymphoma of ovary presenting as peritonitis

1 Department of Radiology, PSG Institute of Medical Sciences, Coimbatore, Tamil Nadu, India
2 Department of HPB and Liver Transplant Surgery, PSG Institute of Medical Sciences, Coimbatore, Tamil Nadu, India
3 Department of Pathology, PSG Institute of Medical Sciences, Coimbatore, Tamil Nadu, India
4 Department of Gastroenterology, PSG Institute of Medical Sciences, Coimbatore, Tamil Nadu, India

Date of Web Publication9-Oct-2015

Correspondence Address:
Salil Pandey
Department of Radiology and Imaging, PSG Institute of Medical Sciences, Peelamedu, Coimbatore-641004, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.140810

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 > Abstract 

Primary ovarian lymphoma is rare, with ovary more commonly involved secondarily in generalized disease. Primary ovarian lymphoma presents as mass in the ovary with chronic symptoms; an acute presentation has not been described previously. A 75-year-old female presented with acute abdomen and features of peritonism. Computed tomography and magnetic resonance imaging demonstrated large mass in left ovary along with infiltration of adjacent sigmoid colon causing perforation and pneumoperitoneum. Few jejunal loops were also involved. Intraoperatively, there was left ovarian mass infiltrating the sigmoid colon with perforation and fecal peritonitis. Distal jejunal loops were adherent to the tumor. The involved sigmoid colon was resected with total abdominal hysterectomy, bilateral salpingo-oophorectomy and resection of adherent jejunal loops. Histopathology revealed ovarian tissue with necrotic neoplasm composed of small-to medium-sized round cells exhibiting nuclear irregularity and scanty cytoplasm, forming discohesive sheets with the neoplasm infiltrating the retroperitoneal remnant tissue and resected bowel. This case highlights an unusual presentation of primary ovarian lymphoma.

Keywords: Infiltration, non-Hodgkin′s lymphoma, ovary, peritonitis

How to cite this article:
Pandey S, Devanand B, John B J, Singh G, Sivanandam S, Leelakrishnan V. High-grade non-Hodgkin's lymphoma of ovary presenting as peritonitis. J Can Res Ther 2015;11:663

How to cite this URL:
Pandey S, Devanand B, John B J, Singh G, Sivanandam S, Leelakrishnan V. High-grade non-Hodgkin's lymphoma of ovary presenting as peritonitis. J Can Res Ther [serial online] 2015 [cited 2020 Jul 10];11:663. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/663/140810

 > Introduction Top

Primary ovarian lymphoma is a rare entity. This is due to the absence of lymphoid tissue within the ovary. Secondary involvement is more likely to be encountered. [1] Primary ovarian lymphoma usually presents as a mass in the ovary with chronic symptoms. We describe a case of non-Hodgkin's lymphoma (NHL) of the ovary presenting as peritonitis. To our knowledge, this is the first case of an acute presentation of primary ovarian lymphoma.

 > Case report Top

A 75-year-old female presented with acute left iliac fossa pain with localized guarding and peritonism. She was undergoing investigations for abdominal pain, constipation, weight loss and anorexia prior to this acute presentation. Serum CA 125 was elevated, measuring 138 U/ml. Serum lactate, cortisol and procalcitonin levels were also elevated. A pelvic pathology was suspected and magnetic resonance imaging (MRI) of the abdomen and pelvis demonstrated a large heterogenous left adnexal mass extending into the central abdomen [Figure 1]. The lesion was hypointense on T1WI and heterogenously hyperintense with internal cystic areas on T2WI and was focally abutting the uterine fundus superiorly [Figure 1]a. There was also wall thickening in the adjacent sigmoid colon with a collection adjacent to sigmoid colon extending superiorly [Figure 1]b. The right ovary appeared normal. The MRI features were suggestive of a left ovarian mass with locoregional infiltration. A definitive treatment including surgical exploration was planned following further staging investigations and the patient was discharged on request. The patient was readmitted shortly with increasing severe abdominal pain and obstipation. A computed tomography (CT) scan obtained during the acute admission demonstrated a large solid hypodense mass anterosuperior to the uterus extending superiorly from the pelvis into the central abdomen. There was infiltration of the sigmoid colon with large wall discontinuity and pneumoperitoneum suggestive of colonic perforation with a resultant large collection in sigmoid mesocolon [Figure 2]a. There was also focal infiltration into an adjacent long loop of jejunum causing wall thickening and tethering of the loop [Figure 2]b. The left ureter was mildly compressed by the large collection. There was no evidence of lymphadenopathy.
Figure 1: (a) Sagittal T2W MRI showed a large heterogenous mass abutting the uterine fundus superiorly, (b) Axial T2W MRI shows focal loss of distinct plane between the lesion and the adjacent sigmoid colon and a paracolonic collection

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Figure 2: (a) Coronal CT shows a hypodense mass infiltrating the sigmoid colon laterally with colonic wall thickening, colonic wall discontinuity and paracolonic collection, (b) Axial CT demonstrates the involvement of jejunal loops at the superior aspect of the lesion

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The patient underwent an exploratory laparotomy prior to which the left ureter was stented. Intraoperative findings revealed a large 10 × 10 cm left ovarian mass infiltrating the sigmoid colon with resultant perforation and fecal peritonitis. Distal jejunal loops were adherent to the tumor. The uterus and right ovary were normal. The involved sigmoid colon was resected along with total abdominal hysterectomy and bilateral salpingo-oophorectomy. A small segment of the jejunum that was adherent to the tumor was also resected. Primary end-to-end anastomosis of the small bowel was performed and a Hartmann's procedure was completed. Following copious washout, the abdomen was closed. The postoperative course was complicated by urosepsis and wound infection. This was managed by removal of the ureteric stent and drainage of the wound along with antibiotics. She was subsequently discharged in a stable condition. Chemotherapy has been planned after an interval period to allow postoperative recovery.

Histopathology showed ovarian tissue with necrotic neoplasm composed of small- to medium-sized round cells exhibiting nuclear irregularity and scanty cytoplasm, forming discohesive sheets [Figure 3]. The neoplasm was infiltrating the retroperitoneal remnant tissue, resected small bowel up to the submucosa, resected large bowel with bulk of the neoplasm in the serosal fat, muscle coat, and submucosa and focal mucosal infiltration. Immunohistochemistry demonstrated the tumor to be  LCA-positive while cytokeratin was negative confirming the tumor as lymphoma. The histopathological diagnosis was of malignant round cell neoplasm consistent with high-grade NHL of the ovary.
Figure 3: (a) Ovary with lymphoma infiltration (40× magnification), (b) Sections show uniform pleomorphic round cell with irregular dark stained nuclei (100× magnification), (c) High power view of the same slide as in Figure 3b

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 > Discussion Top

While primary solid ovarian lymphoma has been described occurring as a rare ovarian tumor, an acute presentation due to sigmoid colon involvement and perforation has not been described before. Secondary involvement of ovary by disseminated lymphoma is common with 7% to 26% incidence [1] , while primary lymphoma arising from the ovary is rare, accounting for 0.5% of NHL and 1.5% of ovarian tumors. [2] The common histologies include high-grade NHLs like Burkitt's lymphoma and diffuse large B cell lymphoma. [2] The ovarian involvement can be unilateral or bilateral with a report of bilateral large masses in Burkitt's lymphoma of ovary. [3] Primary ovarian lymphoma is hypothesized to occur in the lymphocyte within the blood vessels in the ovaries, in the hilum or in relation to corpus luteum. [3] These tumors may also present with ascites, omental deposits or pleural effusion much like the other more common ovarian tumors. [3] Ovarian lymphoma is considered to be an extremely fast growing tumor, with few other ovarian malignancies like immature germ cell tumor or granulocytic sarcoma matching this rate of growth. [3] The criteria for diagnosing primary ovarian lymphoma include (a) the disease to be limited to the ovary, (b) absence of disease in the blood and bone marrow and (c) the extraovarian deposits, if any, should appear at least after few months. [4] The tumor can however be considered to be primary in nature if it has involved the immediate adjacent lymph nodes or adjacent organs. [4] The distinction of primary ovarian lymphoma from the more common ovarian malignancies like epithelial tumors is important since the treatment and prognosis differs. Primary treatment of ovarian lymphoma is chemotherapy, the regimen depending on the histology. Surgery is recommended only in acute emergencies.

Lymphomas in general are known to cause encasement rather than infiltration or disruption of adjacent organs. Ferrozzi et al. studied five cases of NHL of ovary with MRI and noted a predominant non-infiltrative pattern of growth. [5] The extensive infiltration of adjacent organs seen in our patient is an uncommon presentation for this tumor. This infiltrative growth had resulted in sigmoid colon perforation and frank peritonitis. Infiltration into multiple jejunal loops was also noted. Preoperative CT and MRI accurately delineated the tumor arising from the left ovary and delineated involvement of other adjacent structures. Absence of generalized lymphadenopathy was helpful in excluding secondary lymphoma. Preoperative imaging demonstrating involvement of various structures helped in the surgical planning. Positron-emission CT has also been mentioned in regards to preoperative imaging and delineation of the extent of involvement and has been cited as being useful in staging and assessment of therapeutic response. [6] Prognosis of ovarian lymphoma has been described as being variable and depends on the histological subtype, extent of involvement and other clinical variables.

To conclude, primary ovarian lymphoma needs to be considered in the differential diagnosis of solid ovarian masses; this tumor as in our case may rarely infiltrate into adjacent structures, including bowel and present as a surgical emergency necessitating urgent operative intervention with tumor removal or debulking and resection of involved structures, followed by interval chemotherapy.

 > References Top

Hu R, Miao M, Zhang R, Li Y, Li J, Zhu K, et al. Ovary involvement of diffuse large B cell lymphoma. Am J Case Rep 2012;13:96-8.  Back to cited text no. 1
Cyriac S, Srinivas L, Mahajan V, Sundersingh S, Sagar TG. Primary Burkitt's lymphoma of the ovary. Afr J Pediatr Surg 2010;7:120-1.  Back to cited text no. 2
Crawshaw J, Sohaib SA, Wotherspoon A, Shephard JH. Primary non-Hodgkin's lymphoma of the ovaries: Imaging findings. Br J Radiol 2007;80:155-8.  Back to cited text no. 3
Fox H, Langley FA, Govan AD, Hill AS, Bennett MH. Malignant lymphoma presenting as an ovarian tumour: A clinicopathological analysis of 34 cases. Br J Obstet Gynaecol 1988;95:386-90.  Back to cited text no. 4
Ferrozzi F, Tognini G, Bova D, Zuccoli G. Non-Hodgkin Lymphomas of the ovaries: MR Findings. J Comput Assist Tomogr 2000;24:416-20.  Back to cited text no. 5
Komoto D, Nishiyama Y, Yamamoto Y, Monden T, Sasakawa Y, Toyama Y, et al. A case of non-Hodgkin's lymphoma of the ovary: Usefulness of 18F-FDG PET for staging and assessment of the therapeutic response. Ann Nucl Med 2006;20:157-60.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3]


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