A 37-year-old pregnant caucasian woman (3rd pregnancy at sixth month of gestation), at the 33rd week of amenorrhea, arrived in the general emergency room of "Villa Sofia Cervello" Hospital of Palermo, complaining of abdominal pain and vomiting. She reported abdominal pain and distension with bowel disorders (diarrhea alternating with constipation) for about 3 weeks, taking antibiotic for 4 days. From the anamnesis, two previous pregnancies had had a regular course without pathology worthy of note and with spontaneous deliveries. Her past medical and family histories were unremarkable. She had no history of hepatitis, sexually transmitted diseases or other significant illnesses, tobacco or drug use, and neurologic examination was negative. At admission, vital signs were normal, with normal GCS - Glasgow Coma Scale (15/15); moderate dehydration and a diffusely painful abdomen were highlighted. All obstetric parameters were normal: no anomalies emerged from the obstetric visit, with adequate cervicometry. Pregnancy appeared to be in regular evolution: active fetal movements and fetal heartbeats were present, placenta was regular and normoinsert, amniotic fluid was normal. The case was reported in line with the SCARE 2018 criteria.
An abnormal abdominal effusion and overdistension of the intestinal loops with reduced peristalsis was noted and, during an objective exam, a mobile abdominal mass in the right-side seat was noted (approximately 8-10 cm). An abdominal ultrasound highlighted diffused ascites, with involvement of peri-hepatic and peri-splenic areas as well as the pelvis; liver had normal echogenicity, with no focal lesions. In the right pelvic area, in the context of an abundant quantity of fluid effusion, there were some over-stretched loops with reduced peristalsis, uneven echogenicity and indistinct margins. Laboratory workup showed relevant changes in the liver profile (aspartate aminotransferase 236 IU/l; alanine aminotransferase 160 IU/l; alkaline phosphatase 337 U/l; LDH 1171 IU/l), initial anaemia (10.6 g/dl) and minimal reduction of haematocrit (32.5%), white blood cell count of 12,300/mcL (with a lymphocytes reduction); coagulation tests showed an increase in fibrinogen (948 mg%). Serological tests for human immunovirus, hepatitis B, and hepatitis C were negative. Therefore, a diagnosis of "suspected intestinal obstruction" was made and magnetic resonance imaging (MRI) was recommended. MRI revealed, on the left side and in the abdominal centre, the presence of jejunal loops moderately overstretched by liquid with thickened wall of some sections of the small intestine; on the right, the jejunal loops appeared collapsed in a conglomerate appearance, without cleavage planes: here there was thickening of the posterior peritoneum and irregular thickening of the omental apron, delimiting the intestinal mass (Fig. 1).
Differential diagnosis between inflammatory bowel disease and tumor mass (solid and/or hematological) was necessary, and, after gastroenterological, anesthesiological and surgical counseling, surgery was performed. A two-step surgical procedure was decided: first, the "obstetric phase", with a cesarean section, and second, the "surgical phase", focalized on the management of the intestinal mass.
The abdomen was opened with a navel-pubic incision. Approximately 5 L of ascitic fluid were obtained and a sample was sent for cytological examination. A premature baby was delivered with Apgar scores 6/8 at the first and fifth minute.
After closing the uterine breach, the surgical team continued the operating session, highlighting a large neoplastic mass starting from the root of the mesentery that infiltrated the corresponding small loop (Fig. 2). Part of the mesocolon appeared infiltrated by the mass. The mass was not removable, thus bioptic sampling of nodule of the parietal peritoneum, colonic epiploic fringe and part of the large epiploon were performed for extemporaneous examination which posed the hypothesis of intestinal lymphoma.
Immuno-histochemical studies showed positivity for cellular myc oncogene (cMYC) and no B-cell CLL/lymphoma 6 (Bcl6) and B-cell lymphoma 2 (bcl2) translocations were found: diagnosis of sporadic variant BL (not endemic, not immunodeficiency associated) was confirmed (Fig. 3, Fig. 4). Successive MRI showed leptomeningeal lesions and our patient was therefore diagnosed as having stage IV BL based on Ann Arbor staging criteria. She was started on R-Magrath (R-CODOX-M + R-IVAC) protocol at our Onco-hematological Unit and is currently in follow-up, waiting for a marrow transplant. The baby had no health issues and was discharged home with instructions to follow up for routine laboratory studies.