2 Figure 1: Sectioned right and left ovaries, with near-total obliter- ation of the usual follicular cut surface, replaced by the typical tan white appearance of lymphoma. Figure 2: Low power photomicrograph showing sheets of lym- phoma cell replacing the usual histology of ovary (arrow) and inﬁl- trating fallopian tube (arrow heads). was negative. The patient underwent exploratory laparo- tomy, salpingo-oophorectomy, partial omentectomy, and a wedge biopsy of right lobe of liver. The cut surface of the ovaries is shown in Figure 1. Microscopic examination of the ovaries showed diﬀuse inﬁltrates consisting of medium-sized hyperchromatic cells with irregular nuclei, and small multi- ple nucleoli (Figure 2). Mitotic activity was high, and there were areas of increased debris-laden macrophages, giving the appearance of a “starry-sky”. Cell marker study by ﬂow cytometry revealed a monotypic lymphoid cell population, positive for CD10, CD19, CD20, and HLA: DR with kappa light chain restriction. Cytogenetics and molecular genetics showed 46, XX, t(8; 14) (q24; q32). There was no evidence of tumor in the liver or omentum. The bone marrow and CSF were negative for malignant cells. The results of pathology and staging indicated Stage III Burkitt lymphoma (by Murphy staging) [5]. Intention was to treat the patient as per FAB/LMB96 regimen B4, but therapy reductions were made due to com- plications [6]. Her regimen included COP (cyclophospham- ide 300 mg/m2, vincristine 1 mg/m2 once, prednisolone 60 mg/m2/day for 7 days, and intrathecal chemotherapy with methotrexate/hydrocortisone (once)), COPADM1 (cyclo- phosphamide 250 mg/m2 BID for 6 doses, vincristine 2 mg/m2 once, prednisolone 60 mg/m2/day for 5 days, then taper over 3 days, doxorubicin 60 mg/m2 once, methotrexate 3000 mg/m2 once, and methotrexate/hydrocortisone given intrathecally twice), dose-reduced COPADM2 (cyclophos- phamide 250 mg/m2 BID for 6 doses, vincristine 1 mg/m2 Case Reports in Medicine once, prednisolone 60 mg/m2/day for 5 days, then taper over 3 days, doxorubicin 30 mg/m2 once, methotrexate 1500 mg/m2 once, and methotrexate/hydrocortisone given intra- thecally twice), and dose-reduced CYM1 (cytarabine 100 mg/m2 continuous infusion daily for 5 days, methotrexate 1500 mg/m2 once, and intrathecal methotrexate/hydrocor- tisone once and cytarabine/hydrocortisone once). The last cycle of chemotherapy, CYM2, was omitted. Rituximab was given every 2–4 weeks for 5 total doses, starting between COPADM1 and COPADM2, with a cumulative dose of 1875 mg/m2. Our patient developed several complications during and after therapy. These included hypertension and renal insuf- ﬁciency requiring intensive care. She presented with a small bowel obstruction after induction therapy, followed by ab- dominal compartment Syndrome; both required surgical intervention and prolonged administration of parental nutri- tion. She experienced a two-month delay in chemotherapy to allow for healing. The patient had multiple episodes of febrile neutropenia with concomitant sepsis producing delays and dose reduction of chemotherapy. Chemotherapy was dis- continued early due to poor tolerance. Cytopenias persisted for several months oﬀ chemotherapy, but did not require intervention with transfusions or growth factors and sponta- neously resolved at approximately 6 months after rituximab completion. At the time of this report, she has been oﬀ therapy for 22 months and remains in complete remission. 3. Discussion Features of WS include congenital heart disease, hyperten- sion, premature aging of skin, dysmorphic facial features, infantile hypercalcemia, gregarious personality, and mental retardation with Intelligence Quotients ranging from 20 to 106 [7]. A submicroscopic deletion of contiguous genes on the long arm of chromosome 7 is seen in Williams Syndrome. Chromosome 7 is the second most frequently involved chromosome among the cytogenetic abnormali- ties observed in human malignancies after chromosome 8 [8]. The cytogenetic abnormalities in human cells are considered acquired; however, cancer has been reported in persons with constitutional cytogenetic abnormalities involving chromosome 7 [9–15], and it has been speculated that these abnormalities may predispose to malignancies. The literature contains reports of malignancy occurring in Williams Syndrome including ﬁve cases in the pediatric age group and four cases in the adult age group [16]. The pediatric cases include two cases of Burkitt lymphoma (ages 1 and 5 years), a Cutaneous ﬁbrous harmartoma (age 5 years), acute lymphoblastic lymphoma (age 14 years), and astrocytoma in a 5-year-old child [16]. The two prior reported patients with WS and BL toler- ated chemotherapy well, without unexpected complications; one patient received standard-dosing chemotherapy per the same protocol as our patient, but followed the B1 arm which included the second CYM cycle as well as COPADM3, while the other patient received 4 courses of CHOP (cyclophos- phamide, doxorubicin, vincristine and, prednisolone). Due