year old girl presented to our outpatient clinic with a one month history of abdominal pain, vomiting, nausea, early satiety and weight loss of 3
kg. Clinical examination showed pallor and pitting oedema. No palpable abdominal mass was detected clinically but ultrasonography revealed huge bilateral ovarian masses, suggestive of Krukenberg tumor. Initial laboratory findings showed normocytic, normochromic anaemia with a haemoglobin of 93
g/L, a white blood cell count (WBC) of 16.6 x103
, elevated platelets of 484,000/mm3
(neutrophils, lymphocytes and blood film were normal). Total serum proteins as well as albumin were slightly reduced with 43
g/L and 23
g/L respectively, with normal liver and renal function tests. Serum lactate dehydrogenase (LDH) level was 334 UI/L, uric acid 334
μmol/l and ferritin 36
μg/l confirming absence of tumor lysis. Both alpha foeto-protein (AFP) and beta human chorionic gonadotropin (βHCG) were within normal range. The abdominal CT and MRI showed bilateral ovarian masses, peritoneal carcinomatosis, ascites and diffuse and nodular thickening of the gastric and intestinal wall (Figure
Abdominal CT scan with gastric tumor (a) and MRI showing bilateral ovarian masses (b).
Suspecting obstructive endoluminal masses, an upper endoscopy was performed and revealed multiple, large (2 to 3
cm in diameter), raised ulcerated tumors involving the stomach, as well as multiple similar lesions throughout the duodenum and jejunum (Figure
); multiple biopsies were taken from several lesions for further analysis. Histological examination showed diffuse wall infiltration by a medium sized, monotonous, atypical lymphoid cell population with scanty basophilic cytoplasm and a characteristic starry-sky pattern. On immunohistochemistry tumor cells showed positive staining for B-cell-associated antigens (CD 20, CD 10), B cell lymphoma 6 protein (Bcl6), paired box gene 5(PAX 5), surface IgM and IgD as well as monoclonal Kappa light chains. They were negative for B cell leukemia/lymphoma 2(Bcl2) and T-cell marker (CD5). These morphological and immunophenotypical features were consistent with Burkitt’s lymphoma, classified as stage III, Group B according to Murphy Stages. EBV encoded small RNA (EBER) in situ hybridisation showed an 80% consistency with Epstein Barr Virus (EBV). Finally, the typical reciprocal chromosomal translocation involving the proto-oncogene c-MYC on chromosome 8 and the immunoglobulin-gene heavy chain locus on chromosomes 14 [t(8;14)] was found in the tumor cells. Bone marrow aspirate and biopsy, as well as cerebrospinal fluid (CSF) were normal.
Gastric (a) and jejunal (b) lesions on upper endoscopy.
A nasojejunal feeding tube was inserted because of subtotal intestinal obstruction by a tumoral mass at the level of the duodenal bulb. The patient was started on chemotherapy according to POG 9917 protocol, including dexamethasone, methotrexate, cyclophosphamide, vincristin, cytarabin and doxorubicin; intrathecal cytarabin and methotrexate were administered as CNS prophylaxis. To prevent massive tumor lysis syndrome, hyperhydration and two doses of rasburicase were administered before and during the first cycle of chemotherapy. Interestingly, tumor lysis could be easily controlled and a rapid resolution of clinical signs was observed.
According to the protocol, chemotherapy was discontinued after 4
months. An upper endoscopy with biopsies was performed and confirmed remission with absence of lesions.