Diffuse Large B-Cell Lymphoma (DLBCL) is the most frequent subtype of non-Hodgkin's lymphoma (NHL), accounting for 50% of all NHL cases seen at HC-FMUSP. Up to 40-50% of DLBCL cases have extranodal involvement. However, female genital tract involvement is very rare, accounting for less than 0.5% of cases. Spindle/sarcomatoid cell neoplasms usually have mesenchymal histogenesis, however rare cases of aggressive NHL with a spindle-shaped morphological aspect have been recently described, although this variant is still not fully recognized as a specific pathological entity.

To describe a rare case of DLBCL with primary uterine cervix involvement, and sarcomatoid morphological pattern, as well as multi-visceral involvement. We intend to highlight the importance of recognizing this entity in the differential diagnosis of spindle-shaped malignant neoplasms.

A 51-year-old woman, with no previous comorbidities, sought medical service at the HC-FMUSP due to a vulvovaginal tumor with progressive growth over the last 12 months. Gynecological examination showed an ulcerated tumor in the cervix, extending to the vaginal cavity and exteriorizing through the vulvar introitus. Computed tomography showed a 7 cm tumor mass in the cervix and pathological bone fracture of the L3 vertebra. A biopsy of the uterine cervix showed atypical fusiform cell proliferation, inferring a diagnosis of uterine leiomyosarcoma. However, bone biopsy showed proliferation of large atypical lymphoid cells, suggestive of NHL. Immunohistochemical study of both samples revealed: Ki67+ (80%), CD45+, PAX-5+, diffuse staining for CD20+, BCL-6+, CD79a+, CD30+, with negativity for epithelial and mesenchymal lineage markers. 18-FDG-PETCT revealed involvement of lymph nodes, bones, liver, kidney, thyroid, breasts and bulky in the female genital tract. Diagnosis of DLBCL sarcomatoid variant was established and therapy was scheduled with 6 x R-CHOP, 3 cycles of 3 g/m2 methotrexate for CNS prophylaxis and uterine cervix radiotherapy depending on residual uptake on PET-CT after chemotherapy. She is currently after 1st. R-CHOP cycle.

Sarcomatoid variant of DLBCL is rare and already recognized as a morphological variant of DLBCL NOS by WHO-2016 Classification. The explanation for the occurrence of fusiform neoplastic cells remains unclear, but it is believed that tumor stromal fibrosis can justify deformation of the cell membrane of neoplastic cells, giving the sarcomatoid aspect. Its clinical significance, as well as prognostic impact, have not been established, nor has there been any definition of the need for therapy different from conventional treatment based on anthracyclines (R-CHOP).

We described a case of uterine cervix sarcomatoid DLBCL, a rare entity that establishes a differential diagnosis with sarcomas of the female genital tract. Although it is an isolated case, our patient had multivisceral involvement and high-risk IPI, leading us to infer that this morphological variant may be related to greater biological aggressiveness, high tumor burden and poor outcomes, but this needs to be validated with description of other cases and cases series.