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Burkitts’s lymphoma – an atypical presentation
© Ziade et al.; licensee BioMed Central Ltd. 2012
Received: 1 May 2012
Accepted: 30 July 2012
Published: 6 August 2012
In female adolescents and young adults, malignancies of the genital tract are the most frequent type of cancer, closely followed by Hodgkin’s and non-Hodgkin’s lymphomas.
We report an unusual case of sporadic Burkitt’s lymphoma (BL) presenting with massive bilateral ovarian infiltration, peritoneal carcinomatosis and diffuse nodular lesions of the stomach and the intestine mimicking Krukenberg tumor. Diagnostic biopsies were obtained by endoscopy of the upper gastrointestinal tract. With intensive chemotherapy, complete remission was rapidly achieved, without life-threatening tumor lysis syndrome.
Besides metastatic gastric adenocarcinoma, BL is an important differential diagnosis in adolescents presenting with Krukenberg tumor.
KeywordsBurkitt’s lymphoma Gastric Stomach Adolescent Epstein barr virus Krukenberg tumor
Cancer in adolescents and young adults is 2.7 times more common than cancer occurring during the first 15 years of life. In females aged between 15 to 29 years old, malignancies of the genital tract are the most frequent type of cancer (18%), closely followed by lymphomas (17%) . Hodgkin lymphomas account for two–thirds of those cases. The remainder of patients present one of the four subtypes of non-Hodgkin lymphoma (NHL): diffuse large B-cell lymphoma, Burkitt’s lymphoma (BL), lymphoblastic lymphoma or anaplastic large cell lymphoma.
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.
Discussion and conclusion
To the best of our knowledge, this is the first pediatric report of Burkitt’s lymphoma mimicking Krukenberg tumor with synchronous involvement of stomach and ovary, even though ovarian or gastric implants of Burkitt´s lymphoma are a common finding. Similar reports have only been described in adult female patients, where gastric adenocarcinoma was the underlying disease [2, 3].
Krukenberg tumor is generally defined as an ovarian carcinoma that contains a significant component of mucin-filled signet-ring cells typically lying within a cellular stroma derived from the ovarian stroma. It refers to a malignancy with metastasis to other organs, classically the gastrointestinal tract. Today, almost all Krukenberg tumors are thought to be metastatic signet-ring cell carcinoma in the ovary of gastric origin, although rare examples have been interpreted as primary.
Lymphomas represent 26% of all cancers in 15–29 year olds in Europe and the USA. In the 15–19 year-old group, diffuse large B-cell Lymphomas account for the largest proportion of NHL cases (37%) followed by BL (21%) .
Patients with gastrointestinal BL may present with abdominal pain or distension, gastrointestinal bleeding or intestinal obstruction resulting from direct compression of the lumen by an expanding mass or by an intussusception triggered by the intraluminal projection of the tumor mass [5–7]. These acute abdominal symptoms often lead to emergency laparotomies before a diagnosis of Burkitt’s lymphoma can be made .
Spontaneous intestinal perforation is an uncommon complication of BL, even though previously reported in children . Possible explanations for spontaneous gastrointestinal perforation are tumor necrosis, immune suppression and protein malnutrition [9, 10]. Tumor necrosis is probably the most likely reason as perforation usually occurs during or after the first cycle of intensive chemotherapy. Epigastric pain is the first and most common symptom of gastric perforation. A high degree of suspicion should be present at all times, as early diagnosis and emergency surgery are crucial in saving life in the setting of such a complication .
Burkitt’s and B-cell lymphomas in childhood have an excellent overall prognosis regardless of the location (except for primary central nervous system lymphoma), especially when treated with contemporary chemotherapy protocols .
Burkitt lymphoma has to be considered as differential diagnosis in adolescent girls presenting with apparent Krukenberg tumors. Prognosis remains excellent with precise histological and molecular diagnosis, early treatment and careful monitoring and prevention of tumor lysis.
Written consent was obtained from the patient and her parents for publication of this case report.
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