Uterine Extramedullary Plasmacytoma as a Primary Manifestation of Multiple Myeloma

Abstract

The association between plasmacytomas and multiple myeloma (MM) is well-described, and in about one third of the cases of plasmacytoma the additional study will lead to the diagnosis of MM. The finding of plasmacytomas in the genital tract is extremely rare, with sparse cases described in the literature, and these cases pose a challenge regarding the optimal guidance and treatment. This paper describes a case of uterine extramedullary plasmacytoma in a 79-year-old woman with complaints of postmenopausal abnormal uterine bleeding. The complementary study led to the diagnosis of uterine plasmacytoma and, subsequently, of MM. Despite the unfavorable outcome of this case, we consider pertinent to report it because it constitutes a differential diagnosis to be taken into account in the approach of pelvic masses. Palavras-chave► plasmocitoma ► mieloma múltiplo ResumoA associação entre plasmocitomas e mieloma múltiplo (MM) encontra-se bem demonstrada, e em cerca de um terço dos casos de plasmocitoma o estudo adicional conduzirá ao diagnóstico de MM. O achado de plasmocitomas no trato genital é extremamente raro, havendo um número muito limitado de casos descritos na literatura, o que dificulta concluir sobre a melhor forma de orientação e tratamento destes casos. O presente trabalho descreve um caso de plasmocitoma extramedular uterino em mulher de 79 anos estudada por queixas de hemorragia uterina anômala pós-menopáusica. O estudo complementar levou ao diagnóstico de plasmocitoma uterino e, posteriormente, de MM. Apesar do desfecho desfavorável do caso, consideramos pertinente o seu relato por se tratar de um diagnóstico diferencial a levar em consideração na abordagem de massas pélvicas.

Extramedullary plasmacytoma of the uterine cervix arising in an asymptomatic 46-year-old female

“…Approximately 80 % of reported EMPs have been found in the head and neck region, such as the nasal cavity, sinuses, nasopharynx, and larynx, while other less commonly reported sites include the gastrointestinal tract, urogenital tract, skin, lung, and breast ( Codorniz et al, 2017 , Wang et al, 2021 ). There have been few reports of EMPs in the female reproductive system ( Schor et al, 2010 ).…”

“…Solitary plasmacytomas are estimated to represent between 5 and 10 % of all plasma cell dyscrasias ( Dagan et al, 2009 , Kilciksiz et al, 2012 ). The difference between a primary plasmacytoma and multiple myeloma is that the former dyscrasia lacks the systemic findings that are characteristic of multiple myeloma, including lytic bony lesions, hypercalcemia, anemia, and renal disease ( Codorniz et al, 2017 ). It is critical to extensively investigate all findings of a plasma cell population in order to exclude the possibility of multiple myeloma.…”

“…This type of neoplasm rarely develops in the female genital tract. Ten cases describing a primary cervix plasmacytoma have been reported since 1949 ( Codorniz et al, 2017 , Schor et al, 2010 , Wang et al, 2021 , Johansen et al, 1989 , Huang et al, 2008 , Fischer et al, 2003 ). These patients initially presented with vaginal bleeding, coital bleeding, pelvic pain, vaginal discharge, or they were asymptomatic ( Wang et al, 2021 ).…”

Blastic variant of plasma cell myeloma mimicking squamous cell carcinoma of the uterine cervix in a super-morbidly obese female

“…In contrast, MM is characterized by the neoplastic proliferation of plasma cells typically exhibiting bone marrow involvement but extramedullary disease can be present in up to 7–18% of newly diagnosed patients with MM (Bladé et al, 2012). Extramedullary plasmacytomas in the female genital tract are also rare and can present as a solitary plasmacytomas or as part of disseminated MM (Cordorniz et al, 2017).…”

“…The diagnosis of plasma cell myeloma with extramedullary involvement of the genital tract is extremely rare with insight into the diagnosis, treatment and disease course limited to sparse case reports as only twenty-four cases of gynecologic plasmacytomas that have been report with 7 cases of solitary plasmacytomas and 17 cases as either part of disseminated MM with involvement of a gynecologic organ or lacking complete work-up to rule out MM (Cordorniz et al, 2017; Feldman et al, 2017). Given this is a rare clinical condition that can portend a poor clinical prognosis, expeditious diagnosis and treatment with systemic chemotherapy before clinical deterioration may help improve clinical outcome.…”