Endometrial stromal sarcoma

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Endometrial stromal sarcoma
EndometrialStromalSarcomaLowGrade.JPG
Micrograph of a low-grade endometrial stromal sarcoma. H&E stain.
Specialty Oncology, gynecology

Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma (connective tissue) of the endometrium rather than the glands. There are three grades for endometrial stromal tumors, as follows. [1] It was previously known as endolymphatic stromal myosis because of diffuse infiltration of myometrial tissue or the invasion of lymphatic channels. [2]

Contents

Low-grade endometrial stromal sarcoma

Low-grade endometrial stromal sarcoma consists of cells resembling normal proliferative phase endometrium, but with infiltration or vascular invasion. These behave less [3] aggressively, sometimes metastasizing, with cancer stage the best predictor of survival. The cells express estrogen/progesterone-receptors.

Undifferentiated uterine sarcoma

Endometrial Stromal Sarcoma, High-Grade EndometrialStromalSarcoma.JPG
Endometrial Stromal Sarcoma, High-Grade

Undifferentiated uterine sarcoma, or undifferentiated (high-grade) endometrial stromal sarcoma, does not resemble normal endometrial stroma and behaves much more aggressively, frequently metastasizing. The differential includes leukemia, lymphoma, high-grade carcinoma, carcinosarcoma, and differentiated pure sarcomas.

Pathology

Macroscopy

Microscopy

Immunochemistry

Genetic features

A recurrent chromosomal translocation, t(7;17)(p15;q21), occurs in endometrial stromal sarcoma. This translocation leads to the fusion of two polycomb group genes, JAZF1 and JJAZ1, with production of a fusion transcript with anti-apoptotic properties. Even normal endometrial stroma cells express the fusion gene, derived not by translocation, but by the "stitching" together of m-RNAs. Thus, it appears that a pro-survival gene in the normal endometrium is somehow subverted to become pro-neoplastic. [4]

Related Research Articles

Endometrium Inner mucous membrane of the mammalian uterus

The endometrium is the inner epithelial layer, along with its mucous membrane, of the mammalian uterus. It has a basal layer and a functional layer; the functional layer thickens and then is shed during menstruation in humans and some other mammals, including apes, Old World monkeys, some species of bat, the elephant shrew and the Cairo spiny mouse. In most other mammals, the endometrium is reabsorbed in the estrous cycle. During pregnancy, the glands and blood vessels in the endometrium further increase in size and number. Vascular spaces fuse and become interconnected, forming the placenta, which supplies oxygen and nutrition to the embryo and fetus. The speculated presence of an endometrial microbiota has been argued against.

Sarcoma Medical condition

A sarcoma is a malignant tumor, a type of cancer that arises from transformed cells of mesenchymal origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or hematopoietic tissues, and sarcomas can arise in any of these types of tissues. As a result, there are many subtypes of sarcoma, which are classified based on the specific tissue and type of cell from which the tumor originates. Sarcomas are primary connective tissue tumors, meaning that they arise in connective tissues. This is in contrast to secondary connective tissue tumors, which occur when a cancer from elsewhere in the body spreads to the connective tissue. The word sarcoma is derived from the Greek σάρκωμα sarkōma "fleshy excrescence or substance", itself from σάρξsarx meaning "flesh".

Carcinoma A malignancy that develops from epithelial cells

Carcinoma is a malignancy that develops from epithelial cells. Specifically, a carcinoma is a cancer that begins in a tissue that lines the inner or outer surfaces of the body, and that arises from cells originating in the endodermal, mesodermal or ectodermal germ layer during embryogenesis.

Uterine cancer Medical condition

Uterine cancer, also known as womb cancer, includes two types of cancer that develop from the tissues of the uterus. Endometrial cancer forms from the lining of the uterus, and uterine sarcoma forms from the muscles or support tissue of the uterus. Symptoms of endometrial cancer include changes in vaginal bleeding or pain in the pelvis. Symptoms of uterine sarcoma include unusual vaginal bleeding or a mass in the vagina.

Endometrial cancer Uterine cancer that is located in tissues lining the uterus

Endometrial cancer is a cancer that arises from the endometrium. It is the result of the abnormal growth of cells that have the ability to invade or spread to other parts of the body. The first sign is most often vaginal bleeding not associated with a menstrual period. Other symptoms include pain with urination, pain during sexual intercourse, or pelvic pain. Endometrial cancer occurs most commonly after menopause.

Surface epithelial-stromal tumor Medical condition

Surface epithelial-stromal tumors are a class of ovarian neoplasms that may be benign or malignant. Neoplasms in this group are thought to be derived from the ovarian surface epithelium or from ectopic endometrial or Fallopian tube (tubal) tissue. Tumors of this type are also called ovarian adenocarcinoma. This group of tumors accounts for 90% to 95% of all cases of ovarian cancer; however is mainly only found in postmenopausal women with the exception of the United States where 7% of cases occur in women under the age of 40. Serum CA-125 is often elevated but is only 50% accurate so it is not a useful tumor marker to assess the progress of treatment. 75% of women with epithelial ovarian cancer are found within the advanced-stages; however younger patients are more likely to have better prognoses than older patients.

Dermatofibrosarcoma protuberans Medical condition

Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive malignant cutaneous soft-tissue sarcoma. DFSP develops in the connective tissue cells in the middle layer of the skin (dermis). Estimates of the overall occurrence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft tissue sarcomas and 18 percent of all cutaneous soft tissue sarcomas. In the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma.

Liposarcoma Medical condition

Liposarcomas are the most common subtype of soft tissue sarcomas, accounting for at least 20% of all sarcomas in adults. Soft tissue sarcomas are rare neoplasms with over 150 different histological subtypes or forms. Sarcomas, which constitute ~1% of all adult malignancies, are malignant tumors that develop from the stem cells of mesenchymal tissues such as: the osteosarcomas that arise from the osteoprogenitor cells cells of the mature osteocytes in bone tissues; the fibrosarcomas that arise from the precursor cells of the fibrocytes in connective tissues; and the rhabdomyosarcomas that arise from the precursor cells of the myocytes in muscle tissues. Liposarcomas arise from the precursor lipoblasts of the adipocytes in adipose tissues. Adipose tissues are distributed throughout the body, including such sites as the deep and more superficial layers of subcutaneous tissues as well as in less surgically accessible sites like the retroperitoneum and visceral fat inside the abdominal cavity.

Fibrosarcoma Medical condition

Fibrosarcoma is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. In humans it is usually found in males aged 30 to 40. It originates in fibrous tissues of the bone and invades long or flat bones such as the femur, tibia, and mandible. It also involves the periosteum and overlying muscle.

Stromal cells, or mesenchymal stromal cells, are differentiating cells found in abundance within bone marrow but can also be seen all around the body. Stromal cells can become connective tissue cells of any organ, for example in the uterine mucosa (endometrium), prostate, bone marrow, lymph node and the ovary. They are cells that support the function of the parenchymal cells of that organ. The most common stromal cells include fibroblasts and pericytes. The term stromal comes from Latin stromat-, "bed covering", and Ancient Greek στρῶμα, strôma, "bed".

Nodular fasciitis Medical condition

Nodular fasciitis (NF) is a benign, soft tissue tumor composed of myofibroblasts that typically occurs in subcutaneous tissue, fascia, and/or muscles. The literature sometimes titles rare NF variants according to their tissue locations. The most frequently used and important of these are: cranial fasciitis and intravascular fasciitis. In 2020, the World Health Organization classified nodular fasciitis as in the category of benign fibroblastic/myofibroblastic tumors. NF is the most common of the benign fibroblastic proliferative tumors of soft tissue and exceeds in frequency any other tumor or tumor-like lesion in this group of tumors.

The uterine sarcomas form a group of malignant tumors that arises from the smooth muscle or connective tissue of the uterus.

Endometrial intraepithelial neoplasia (EIN) is a premalignant lesion of the uterine lining that predisposes to endometrioid endometrial adenocarcinoma. It is composed of a collection of abnormal endometrial cells, arising from the glands that line the uterus, which have a tendency over time to progress to the most common form of uterine cancer—endometrial adenocarcinoma, endometrioid type.

Mixed Müllerian tumor Medical condition

A malignant mixed Müllerian tumor, also known as malignant mixed mesodermal tumor (MMMT) is a cancer found in the uterus, the ovaries, the fallopian tubes and other parts of the body that contains both carcinomatous and sarcomatous components. It is divided into two types, homologous and a heterologous type. MMMT account for between two and five percent of all tumors derived from the body of the uterus, and are found predominantly in postmenopausal women with an average age of 66 years. Risk factors are similar to those of adenocarcinomas and include obesity, exogenous estrogen therapies, and nulliparity. Less well-understood but potential risk factors include tamoxifen therapy and pelvic irradiation.

Alveolar soft part sarcoma Medical condition

Alveolar soft part sarcoma, abbreviated ASPS, is a very rare type of soft-tissue sarcoma, that grows slowly and whose cell of origin is unknown.

Uterine clear-cell carcinoma (CC) is a rare form of endometrial cancer with distinct morphological features on pathology; it is aggressive and has high recurrence rate. Like uterine papillary serous carcinoma CC does not develop from endometrial hyperplasia and is not hormone sensitive, rather it arises from an atrophic endometrium. Such lesions belong to the type II endometrial cancers.

Desmoplasia

In medicine, desmoplasia is the growth of fibrous or connective tissue. It is also called desmoplastic reaction to emphasize that it is secondary to an insult. Desmoplasia may occur around a neoplasm, causing dense fibrosis around the tumor, or scar tissue (adhesions) within the abdomen after abdominal surgery.

Mammary-type myofibroblastoma Medical condition

Mammary-type myofibroblastoma (MFB), also named mammary and extramammary myofibroblastoma, was first termed myofibrolastoma of the breast, or, more simply, either mammary myofibroblastoma (MMFB) or just myofibroblastoma. The change in this terminology occurred because the initial 1987 study and many subsequent studies found this tumor only in breast tissue. However, a 2001 study followed by numerous reports found tumors with the microscopic histopathology and other key features of mammary MFB in a wide range of organs and tissues. Further complicating the issue, early studies on MFB classified it as one of various types of spindle cell tumors that, except for MFB, were ill-defined. These other tumors, which have often been named interchangeably in different reports, are: myelofibroblastoma, benign spindle cell tumor, fibroma, spindle cell lipoma, myogenic stromal tumor, and solitary stromal tumor. Finally, studies suggest that spindle cell lipoma and cellular angiofibroma are variants of MFB. Here, the latter two tumors are tentatively classified as MFB variants but otherwise MFB is described as it is more strictly defined in most recent publications. The World Health Organization, 2020, classified mammary type myofibroblastoma tumors and myofibroblastoma tumors as separate tumor forms within the category of fibroblastic and myofibroblastic tumors.

Endometrial cups form during pregnancy in mares and are the source of equine chorionic gonadotropin (eCG) and a placenta-associated structure, which is derived from the fetus. Their purpose is to increase the immunological tolerance of the mare in order to protect the developing foal.

Proliferative fasciitis and proliferative myositis (PF/PM) are rare benign soft tissue lesions that increase in size over several weeks and often regress over the ensuing 1–3 months. The lesions in PF/PM are typically obvious tumors or swellings. Historically, many studies had grouped the two descriptive forms of PF/PM as similar disorders with the exception that proliferative fasciitis occurs in subcutaneous tissues while proliferative myositis occurs in muscle tissues. In 2020, the World Health Organization agreed with this view and defined these lesions as virtually identical disorders termed proliferative fasciitis/proliferative myositis or proliferative fasciitis and proliferative myositis. The Organization also classified them as one of the various forms of the fibroblastic and myofibroblastic tumors.

References

  1. Sternberg's Diagnostic Surgical Pathology, 5th edition, p. 2242-2245.
  2. Kumar V, Abbas A, Fausto N, Aster J (2010). Robbins and Cotran Pathologic Basis of Disease. 8th edition. Philadelphia: Elsevier Saunders
  3. "What is Uterine Sarcoma?".
  4. Li H, et al. (2009). "Gene fusion and RNA trans-splicing in normal and neoplastic cells". Cell Cycle. 8 (2): 218–222. doi: 10.4161/cc.8.2.7358 . PMID   19158498.
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