Myometrial Myxoidosis: A Report of 2 Cases of a Distinctive Type of Secondary Myometrial Hypertrophy in Patients With Lupus Erythematosus

Veras, Emanuela; Junkins‐Hopkins, Jacqueline M.; Marinis, Spyridon I.; Vang, Russell

doi:10.1097/pgp.0b013e3181846c87

Cited by 10 publications

(9 citation statements)

References 37 publications

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“…These tumors can behave in a malignant manner if large, associated with abundant myxoid change, brisk mitotic rate, or tumor cell necrosis. 80 Rarely, myxoid change can occur in the myometrium in patients with lupus erythematosus (with secondary muscle hypertrophy, termed as "myxoidosis") 82 or neurofibromatosis, type I 83 forming variably circumscribed nodules. This phenomenon may raise problems in the differential diagnosis with a "low-grade" myxoid smooth muscle tumor in a curettage/biopsy specimen but not in hysterectomy specimens.…”

Section: Tumor Cell Necrosismentioning

confidence: 99%

Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia

Oliva

2016

Modern Pathology

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Among mesenchymal tumors of the uterus, smooth muscle neoplasms are most common. The wide morphologic spectrum, especially within the category of leiomyomas, is responsible for diagnostic problems more frequently with leiomyosarcoma (including mitotically active, apoplectic, and leiomyoma with bizarre nuclei) but also with endometrial stromal tumors. In the former scenario, clinical information, gross appearance as well as strict utilization of morphologic criteria including cytologic atypia, mitotic activity, and tumor cell necrosis are clues in establishing the correct diagnosis. It is important to keep in mind that mitotic rate thresholds vary for the different subtypes of leiomyosarcoma. Of note, p16 should be used with caution in supporting a diagnosis of leiomyosarcoma as it is often positive in leiomyomas with bizarre nuclei and leiomyomas with apoplectic change (in the latter most frequently and more intense near areas of necrosis). MED12 mutations have also a very limited role in this differential diagnosis. Endometrial stromal tumors are by far, less common than smooth muscle tumors, but can be confused with leiomyosarcomas if they are associated with an undifferentiated uterine sarcoma and the low-grade component is overlooked or they have a myxoid/fibroblastic morphology. The differential diagnosis may be confounded if the latter is associated with a high-grade endometrial stromal sarcoma. It is important to highlight that CD10 is not a reliable marker in these differentials and should be used as a part of a panel of antibodies that also includes desmin and h-caldesmon. Two other recently categorized tumors in the uterus that merit special mention are PEComa and inflammatory myofibroblastic tumor as they enter in the differential diagnosis of smooth muscle tumors. PEComa may be part of the tuberous sclerosis syndrome and may show either a predominantly epithelioid or spindle morphology or combination thereof. Rarely, it may contain melanin pigment. There is variable positivity for HMB-45, MelanA, MiTF, and CathepsinK, and some tumors have been shown to express TFE-3 especially when associated with "clear cell" morphology. Patients with adverse outcome have tumors with ≥ 2 of the following features: ≥ 5 cm, infiltration, high-grade cytologic features, mitotic rate ≥ 1/50 high-power fields, necrosis, or lymphovascular invasion. Inflammatory myofibroblastic tumor is important to recognize as it often mimics myxoid smooth muscle tumors, either benign or malignant. The presence of an associated lymphoplasmacytic infiltrate should alert to that possibility and ALK studies (immunostain or FISH) are helpful in establishing this diagnosis. These tumors can behave in a malignant manner if large, associated with abundant myxoid change, brisk mitotic rate or show tumor cell necrosis.

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Section: Tumor Cell Necrosismentioning

confidence: 99%

Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia

Oliva

2016

Modern Pathology

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“…For nearly all of these lesions, the presence of neoplastic cells embedded in the myxoid stroma distinguishes them from the organizing mucin encountered in this case. The exception is the diffuse myxoid change seen in 2 patients with lupus, but this lesion is characterized by increased myxoid material between smooth muscle cells of the myometrium and has no real morphologic overlap with our case (17).…”

Section: Pseudomyxoma Endometriimentioning

confidence: 64%

“…Myxomas (12), myxoid liposarcoma (13), myxoid chondrosarcoma (14), myxoid malignant peripheral nerve sheath tumor (15), and ''myxoid malignant fibrous histiocytoma'' (16) have also been reported in the uterus. Six cases of presumptively nonneoplastic multifocal or diffuse myometrial myxoid change have also been reported, 2 in patients with systemic lupus erythematosus, and 2 in patients with neurofibromatosis (17)(18)(19). For nearly all of these lesions, the presence of neoplastic cells embedded in the myxoid stroma distinguishes them from the organizing mucin encountered in this case.…”

Section: Pseudomyxoma Endometriimentioning

confidence: 96%

“Pseudomyxoma Endometrii”

Shaw

Kokh²,

Ioffe³

et al. 2015

International Journal of Gynecological Pathology

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Low-grade appendiceal mucinous neoplasms (LAMNs) are commonly associated with deposition of mucin, with or without admixed low-grade epithelium, on peritoneal surfaces (pseudomyxoma peritonei). We describe a very rare presentation of LAMN as extensive mucin deposition in the endometrium of a 43-yr-old woman initially mistaken for a primary uterine myxoid neoplasm. The patient underwent endometrial curettage that demonstrated abundant myxoid/mucoid material interspersed with small vessels, bland eosinophilic spindled cells, scattered foci of typical endometrial stroma, and occasional endometrioid glands. The endometrial stroma was positive for CD10, and the eosinophilic spindled cells were positive for actin. The lesion was interpreted as "myxoid/mucinous neoplasm, most likely of smooth muscle/endometrial stromal origin." Subsequent laparotomy revealed peritoneal mucin in the anterior cul-de-sac and a dilated appendix. Pathologic review confirmed appendiceal LAMN and multifocal peritoneal mucinosis. The uterus contained scant residual mucoid material. On review of all pathologic material at our institution, the endometrial lesion was consistent with organizing mucin derived from the LAMN with entrapped benign endometrium. "Pseudomyxoma endometrii" is readily mistaken for a primary uterine myxoid neoplasm, particularly myxoid endometrial stromal tumor. A key to diagnosis is recognition that the material is mucin rather than myxoid stroma. This is evidenced by the absence of embedded stromal cells and presence of myofibroblastic, vascular, and macrophage infiltration associated with organization. Epithelium containing goblet cells is an important clue if present. The presence of rare endometrial glands within the endometrial stroma suggests that the latter is entrapped rather than neoplastic.

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“…Myometrial myxoidosis is a newly recognized form of non-neoplastic myometrial change, which was first described by Veras et al in 2009. 3 It is viewed as extracellular accumulations of mucin in the myometrial wall. 3 We report a long-standing myxoid degeneration with chronic myometrial inflammation on a lupus background.…”

Section: Discussionmentioning

confidence: 99%

“…2 Myometrial myxoidosis is a pseudo-neoplastic myxoid reaction of the myometrium and is believed to cause myometrial thickening due to abnormal accumulation of myxoid substances. 3 Differential diagnosis of such non-neoplastic lesions from other malignant uterine tumors seems plausible. In addition, its association with other systemic pathological conditions that may contribute to pathogenesis may be of considerable concern.…”

mentioning

confidence: 99%

Myometrial myxoidosis in a patient with systemic lupus erythematosus

Ghamdi

2021

Ann Saudi Med

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Myometrium myxoidosis is new terminology to describe a non-neo-plastic process of extracellular mucinous accumulation in the myometrium wall of the uterus. We report a rare association of myometrial myxoidosis with lupus erythematosus in a 35-year old woman with a history of leiomyoma. At presentation, this case was diagnosed as a pelvic abscess and treated with specific antibiotherapy, and then discharged after clinical improvement. One week later, after recurrence of the symptoms, the patient underwent hysterectomy with bilateral salpingo-oophorectomy and appendectomy with anterior abdominal soft tissue part resection. Pathological analysis revealed diffuse hypo-cellular myxoid areas intersecting the smooth muscle layer of the uterus and cervix in addition to a focal area in the interstitium of smooth muscles of the appendix and soft tissue of the anterior abdominal wall. This case emphasizes the importance of distinguishing between myxoid neoplastic and non-neoplastic lesions with myxoid changes. SIMILAR CASES PUBLISHED: To our knowledge, there are only two similar cases reported.

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Myometrial Myxoidosis: A Report of 2 Cases of a Distinctive Type of Secondary Myometrial Hypertrophy in Patients With Lupus Erythematosus

Cited by 10 publications

References 37 publications

Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia

Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia

“Pseudomyxoma Endometrii”

Myometrial myxoidosis in a patient with systemic lupus erythematosus

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