Primary Granulocytic Sarcoma of Cervix (PGSC): Review and Analysis of 13 Cases

Introduction: Primary granulocytic sarcoma (PGS) is an extramedullary tumor of immature granulocytic cells without prior evidence of leukemia，it often develops to AML by months to years, carrying a poor prognosis. PGS that occur in cervix are rare. The case reports or retrospective case series about it are scarce too. The tumor may involve in any organ and there is no abnormality in bone marrow, so clinicians always confronted with diagnostic and therapeutic challenges. This abstract aims at assisting physicians precisely diagnosing the PGSC as soon as possible and may indicate the timely appropriate therapy which can reduce the risk progressing to AML.

Methods: we retrospectively reviewed the database of PubMed and CNKI between January 2003 to May 2014, using the term “Myeloid sarcoma” or “Granulocytic sarcoma” or “Extramedullary Myeloid Cell Tumor” and “cervix”; we also evaluated potentially relevant reports referenced in retrospective case series and a case of PGSC diagnosed in our hospital. The patients’ age, initial and accompanied symptoms, pathological section and Immunohistochemical staining of the tumor, as well as the treatment and prognosis were analyzed.

Results: There are 13 cases of the PGSC, 11 of which reported in China and 2 reported abroad. Their age range from 28 to 46 years; These PGSC cases had various presentations in the form of irregular vaginal bleeding (11/13), pain of underbelly (2/13),with other accompanied symptoms such as dyspareunia and increasing of leucorrhea. All the cases made definite diagnosis through pathology and a series of appropriate immunohistochemical panel of antibodies that includes myeloperoxidase, lysozyme,CD68,CD43,CD34,CD20,CD3,CD117,CD45;the tumor cells of all the 13 cases showed positive immunohistochemical reactivity with MPO and at least one of the myelocytic antigens (HLA-DR,CD34,CD117, etc) was expressed. The following antibodies: CD20, CD3 did not react in the neoplastic cells. Several of the cases be mistaken for lymphoma as the two may be indistinguishable on routine hematoxylin and eosin staining; PGS cells, nuclei are slightly smaller with more finely dispersed chromatin, and some cells may show recognizable myeloid differentiation. Most of them received systemic chemotherapy associated with AML, the induce chemotherapy included DA (6/14), IA (2/14), HA (1/14); 2 of the 14 patients received paclitaxel (TAX) combined with chemotherapy;3 patients underwent combination therapy united with operations, radiotherapy and chemotherapy. To prevent the leukemia localization of the central nervous system, the patient in our hospital received intrathecal injection (MTX+Ara-c+Dex), others in the reviewed literature not seen. She has finished her first combined chemotherapy and local radiotherapy of pelvic cavity. PET-CT after the fifth chemotherapy showed complete resolution of the disease site. After 6 months, BM examination showed progression to AML-2a. Leukemia cells were detected quantification of the WT1 and ABL showed 2.49×10^4;and ABL1 7.52×10^4.Leukemia cells were showed negative for AML/ETO1 and Flt-3. Other patients reported in the literature kept a leukemia-free survival state during the follow-up period ranged from 2 months to 6 years.

Conclusions: Based on our review of the literature and the case diagnosed in our hospital, the majority of the PGSC patients are presented with vaginal or postcoital bleeding. When the tumor mass is observed in an unusual location without abnormal of hematology, many patients are misdiagnosed or never diagnosed. To make a precise diagnosis, the use of a series of appropriate immunohistochemical panel of antibodies that includes myeloperoxidase, lysozyme, CD68, CD43, CD34, CD20, CD3, however, can successfully identify the vast majority of PGSC. Besides, examinations such as BM aspiration, Cytogenetic, FISH tests and fusion gene were required to make sure whether it is accompanied with AML and monitor the minimal residual disease or indicate a poor risk category. As a systemic disease, combination therapy should united with operations, radiotherapy and chemotherapy, especially the early and intensive chemotherapy can improve prognosis and help to prevent or delay AML, HSCT is also recommended. To prevent the leukemia localization of the central nervous system, intrathecal injection may aid to patients’ longer survival.