MRI image of large bulky cervix with posterior mass contiguous with wall of rectum. 

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... non-Hodgkin lymphoma (NHL) of the genital tract is a rare fi nding accounting for less than 1% of all extra-nodal disease, with that of the cervix accounting for 0.2 – 0.6% (Chan et al., 2005). Most patients present at a mean age of 46 with vaginal bleeding, a large, bulky cervix on exam, and without the common B symptoms of lymphoma. Cervical cytology is not always helpful as the disease originates from the cervical stroma (Chan et al., 2005). Approximately 70% are diagnosed at Ann Arbor stage I, 22% at stage II, and 8% stage III and above. To date, approximately 120 cases of primary extra-nodal cervical lymphoma have been reported. Diffuse large B-cell lymphoma (DLBCL) subtype is most commonly found at a rate of 30% (Anagnostopoulos et al., 2013). We describe an interesting case by virtue of the unique presentation of this disease and the extent of pelvic organ involvement. A 22 year old G1P1 Hispanic female presented with complaints of left lower extremity edema and rapidly increased abdominal girth with pelvic pain over the past two months. She reported a history of normal menses, except for the most recent which was prolonged. She had no history of abnormal pap smears. Obstetrical history noted a single spontaneous vaginal delivery at term in 2012. Her medical and surgical history was signi fi cant for the report of two negative lymph node biopsies (inguinal and cervical neck) performed in Puerto Rico for a reason unknown to the patient. Physical exam revealed the left lower extremity with +1 pedal and ankle edema, and trace lower leg edema. Right leg was normal, with no calf tenderness bilaterally. Pelvic exam revealed a fi rm cervix with no gross lesions, upper one third of vagina was fi rm, indurated nearly circumferentially. Left parametria was fi rm and 12 cm uterus fi xed to left sidewall. Her initial work up was negative for lower extremity deep venous thrombosis. Presenting laboratory values showed a white blood count 9.7 × 10 3 /mm 3 , hemoglobin 10.5 g/dL, platelets 232 × 10 3 /mm 3 , and a creatinine of 5.26 mg/dL. A pelvic CT scan without contrast revealed marked thickening of the cervix and lower uterine segment, bilateral obstructive hydronephrosis and hydroureter, with circumferential thickening of the bladder and in fl ammatory changes in the left inguinal and femoral region of the left upper thigh. Nuclear renal scan revealed complete absence of left kidney function. A percutaneous nephrostomy tube was placed in the right kidney to treat the obstruction. An endometrial biopsy and pap smear were also performed and noted secretory endometrium and atypical squamous cells of undetermined signi fi cance, human papilloma virus negative, respectively. Colposcopy revealed no lesions and two biopsies of the transformation zone and ECC displayed no evidence of malignancy. Once her creatinine normalized an MRI with contrast was obtained, revealing a large mass involving the posterior portion of the cervix ex- tending upward toward the body of the uterus for a distance of 6.1 cm and measured 4.1 cm in transverse dimension demonstrating a slight increase in T2 signal with minimal enhancement. The mass extended laterally to the pelvic sidewall, posteriorly into the presacral space and inferiorly making it contiguous with the left lateral wall of the rectum. Bulky left internal iliac adenopathy was also present (Fig. 1). An exam under anesthesia with loop electrosurgical excisional pro- cedure and vaginal biopsies was performed. Histology revealed diffuse in fi ltration by large neoplastic lymphocytes. Immunostaining was dif- fusely positive for pan B-cell markers CD20 and PAX5 with lymphoma cells showing an activated B-cell immunophenotype (CD10 negative; BCL6 and MUM1 positive) using the Hans algorithm. The proliferation index as determined by Ki67 staining was estimated to be 70 – 80% (Figs. 2, 3). These fi ndings led to the diagnosis of DLBCL. PET scan displayed FDG activity in the known pelvic mass as well as posterior direct extension into the adjacent rectal wall. Nodal masses were noted lateral to the left side of uterine body and right internal iliac. Bone marrow biopsy was performed, but specimen was insuf fi cient for accurate evaluation, staging her lymphoma at IIE. The patient started chemotherapy within a few weeks of diagnosis. Treatment consisted of rituximab, cyclophosphamide, hydroxydaunomycin, Oncovin and prednisone (R-CHOP). Due to the concern of rectal involvement a gastroenterology workup was initiated. A lower endoscopic ultrasound revealed a hypoechoic mass in the anterior perirectal space. Sonographic evidence suggested invasion into the muscularis propria between 12 and 18 cm from the anal verge. Diagnostic laparoscopy was performed with the intent of performing a diverting colostomy, but due to dif fi culty mobilizing the descending colon secondary to dense adhesions, a loop ileostomy was performed in order to provide prophylaxis against possible bowel perforation at chemotherapy induction. Our patient is still undergoing chemotherapy, but has had signi fi cant delays in timing of treatments due to numerous hospital admissions for neutropenic fever, pain, and urinary infections. A CT scan during a hos- pitalization, approximately 3 months after her diagnosis and third cycle of R-CHOP showed signi fi cant response as judged by the size and homo- geneity of the uterus and cervix. To date she has received 5 of 6 total cycles of R-CHOP. PET/CT after cycle 5 showed no evidence of residual metabolically active tumor in the abdomen or pelvis and no sites of FDG activity to suggest recurrence of nodal or extranodal lymphoma. There is no current plan for radiation treatment. This case is very unique and challenging due to its rarity, metastatic location and patient age. Only one case of DLBCL of the cervix presenting with bilateral hydronephrosis in an 82 year old exists in the literature and with no mention of direct rectal invasion (Novotny et al., 2011). This patient is the youngest documented to be affected by the disease (Anagnostopoulos et al., 2013). The diagnostic challenge is revealed by the similar presentation to squamous cell carcinoma of the cervix with vaginal bleeding, fi xed, en- larged uterus and cervix as well as possible parametrial in fi ltration (Durson et al., 2005). It is very important to differentiate the etiology of the tumor as treatment options are very different between squamous cell/adenocarcinoma of the cervix versus B cell lymphoma, as surgery and radiation are the mainstays of the former and immuno/chemotherapy and at times radiation are of the latter. Cervical lymphoma is differentiat- ed by its lack of involvement of squamous epithelium, therefore making cytology smears generally non-diagnostic. This malignancy arises from the cervical stroma (Anagnostopoulos et al., 2013), and generally requires a deep cervical biopsy for diagnosis. Histopathologic evaluation must be carried out for de fi nitive diagnosis of speci fi c cell type of lymphoma, as was done in our patient. Given the rare nature of cervical lymphoma, there is no consensus regarding management and all therapies have been extrapolated from other types of non-gynecologic lymphoma. Successful treatments have been reported with the regimen of cyclophosphamide, hydroxydaunomycin, Oncovin and prednisone (CHOP), new immu- notherapy rituximab in addition to CHOP (R-CHOP), radiation and surgery. Rituximab, a chimeric monoclonal antibody directed against the CD20 antigen on B-cells, in addition to CHOP has shown, in numerous randomized control trials, to have better overall survival than CHOP alone for non-gynecologic DLBCL (Coif fi er et al., 2002). Looking at treatment options speci fi cally for cervical lymphoma, the largest review of cases to date, consisting of 118, showed that 9.2% had surgery only, 16.8% had chemotherapy only, 10.9% had radiotherapy only and the remaining had multi-modal treatment. Rituximab was utilized in 11.7% of cases. No evidence of recurrence was found in 85.2% of patients at a mean follow-up of 40.5 months, with 10 patients died from their disease within 40 months (Anagnostopoulos et al., 2013). Although there is no gold standard for treatment, most treatments involve chemotherapy and have led to acceptable survival data. Another interesting challenge in our patient is her ...