endometrial stromal sarcoma

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 [sahr-ko´mah] (pl. sarcomas, sarco´mata.)
A tumor, often highly malignant, composed of cells derived from connective tissue such as bone, cartilage, muscle, blood vessel, or lymphoid tissue; sarcomas usually develop rapidly and metastasize through the lymph channels. Different types are named for the specific tissue they affect: fibrosarcoma in fibrous connective tissue; lymphosarcoma in lymphoid tissues; osteosarcoma in bone; chondrosarcoma in cartilage; rhabdosarcoma in muscle; and liposarcoma in fat cells. adj., adj sarco´matous.
Abernethy's sarcoma a malignant fatty tumor occurring mainly on the trunk.
alveolar soft part sarcoma one with a reticulated fibrous stroma enclosing groups of sarcoma cells enclosed in alveoli walled with connective tissue.
botryoid sarcoma (sarcoma botryoi´des) an embryonal rhabdomyosarcoma arising in submucosal tissue, usually in the upper vagina, cervix uteri, or bladder neck in young children and infants, presenting grossly as a polypoid grapelike structure.
endometrial stromal sarcoma a pale, polypoid, fleshy, malignant tumor of the endometrial stroma.
Ewing's sarcoma a malignant tumor of the bone that arises in medullary tissue, usually found in cylindrical bones, with pain, fever, and leukocytosis as prominent symptoms; called also Ewing's tumor.
giant cell sarcoma a malignant form of giant cell tumor of bone.
granulocytic sarcoma chloroma.
immunoblastic sarcoma of B cells an aggressive B-cell lymphoma believed to arise from transformed interfollicular B lymphocytes, which in many cases is associated with a preexisting immunologic disorder, e.g., Sjögren's syndrome, systemic lupus erythematosus, or Hashimoto's thyroiditis, or with an immunocompromised state.
immunoblastic sarcoma of T cells a group of T-cell lymphomas comprising tumors derived from T lymphocytes in the paracortical area arising from a mixture of small lymphocytes and many large transformed cells; the latter are characterized by one or more small but distinctly eosinophilic nucleoli.
Kaposi sarcoma a multicentric, malignant neoplastic vascular proliferation characterized by bluish red cutaneous nodules, usually on the lower extremities, most often on the toes or feet; the nodules slowly increase in size and number and spread to more proximal sites. Tumors often remain confined to skin and subcutaneous tissue, but widespread visceral involvement may occur. The condition occurs endemically in certain parts of Central Africa and Central and Eastern Europe, and a particularly virulent and disseminated form occurs in immunocompromised patients such as transplant recipients and those with acquired immunodeficiency syndrome. Human herpesvirus 8 has been implicated as a causative agent.
lymphatic sarcoma diffuse lymphoma.
osteogenic sarcoma a malignant primary tumor of bone composed of a malignant connective tissue stroma with evidence of osteoid, bone, and/or cartilage formation; depending upon the dominant component, classified as osteoblastic, fibroblastic, or chondroblastic.
pseudo–Kaposi sarcoma unilateral subacute to chronic dermatitis, often with postinflammatory hyperpigmentation, occurring in association with underlying arteriovenous fistula, which closely resembles Kaposi sarcoma both clinically and histologically.
reticulum cell sarcoma histiocytic lymphoma.

en·do·me·tri·al stro·mal sar·co·ma

a term sometimes used for a relatively rare sarcoma believed to be a form of endometriosis in which the lesions form multiple foci in the myometrium and in vascular spaces in other sites, and which consist of histologic and cytologic elements that resemble those of the endometrial stroma.
A popular term for the growth pattern of the rare (0.2% of female genital tract tumours, more common in women under age 50) intravascular leiomyomatosis, an indolent uterine stromal tumour which grows extensively within vessels, clinically characterised by abnormal uterine bleeding, pelvic pain and uterine enlargement
Management Cytoreductive surgery, i.e., bilateral annexectomy and total hysterectomy; medical management with adjuvant progestin therapy is currently the most effective treatment for curing and preventing local recurrence. Aromatase inhibitors may be effective
Behaviour Local recurrence occurs in 70% of cases; metastasis is rare even after suboptimal surgical excision
Prognosis ± 85% 15-year survival, despite metastases

en·do·me·tri·al stro·mal sar·co·ma

(en'dō-mē'trē-ăl strō'măl sahr-kō'mă)
A term sometimes used for a relatively rare sarcoma believed to be a form of endometriosis in which the lesions form multiple foci in the myometrium and in vascular spaces in other sites, and which consist of histologic and cytologic elements that resemble those of the endometrial stroma.
References in periodicals archive ?
A, Fragments of low-grade endometrial stromal sarcoma in an endometrial curettage specimen.
Endometrial stromal sarcomas (ESSs) are rare tumors, constituting about 2% to 4% of all uterine malignant tumors.
A very late recurrence of a formerly misdiagnosed low grade endometrial stromal sarcoma metastasized to the colon.
Genomic landscape of endometrial stromal sarcoma of uterus.
Perivascular Epithelioid Cell Tumor Versus LMS and Endometrial Stromal Sarcoma.
Therefore, it is important for the surgical pathologist to recognize this rare entity and differentiate it from other lesions, particularly tumors of intermediate malignant potential, such as low-grade endometrial stromal sarcoma or endometrioid carcinoma with sex-cordlike features and the highly aggressive adenosarcoma, carcinosarcoma, and metastatic ovarian sex-cord stromal tumor by thorough sampling, use of a proper IHC panel, and awareness of the patient's complete clinical picture.
Rabban and coworkers (8) detected no ALK-1 staining among 7 uterine leiomyomas, 6 uterine leiomyosarcomas, 4 uterine carcinosarcomas, 4 endometrial stromal sarcomas, and 1 uterine lymphangiomyomatosis.
The tumors of one group are morphologically similar to low-grade endometrial stromal sarcomas with diffuse HMB-45 expression and focal expression of smooth muscle markers.
Adenomyosis with sparse glands: a potential mimic of low-grade endometrial stromal sarcoma.
In this article, we present an endometrial stromal sarcoma of the rectosigmoid colon arising in extragonadal endometriosis.
Homologous sarcoma has the typical appearance of spindle-cell sarcoma, resembling high-grade endometrial stromal sarcoma, leiomyosarcoma, fibrosarcoma, malignant fibrous histiocytoma, or undifferentiated sarcoma.