Ebook Sarcoma of the female genitalia (Vol 1): Part 2
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Ebook Sarcoma of the female genitalia (Vol 1): Part 2
Giinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2h sex cord-like elements (ESTSCLE), uterine tumor resembling ovarian sex-cord tumor (UTROSCT) and similar tumorsIntroductionThe current WHO Classification (36) divides EST and similar neoplasms into ESN, LOESS, HG-ESS and ƯƯS. UTROSCT also belong lo the EST family of tumors. ESN are benign stromal t Ebook Sarcoma of the female genitalia (Vol 1): Part 2umors. There are special forms of EST with different types of differentiation, like the endometrial stromal tumor with endometrioid glands, mixed endoEbook Sarcoma of the female genitalia (Vol 1): Part 2
metrial stromal tumors and smooth muscle tumors as well as endometrial stromal tumors with sex cord-like elements (ESTSCLE). and uterine tumors resembGiinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2sed in separate chapters (Chapters 4 and 5).3.1Endometrial Stromal nodule3.1.1Uterine endometrial stromal noduleGeneral, pathogenesis, pathological-anatomical featuresESN is a benign EST with histological features unambiguously reminiscent of endometrial stroma in the proliferation phase. Overall, E Ebook Sarcoma of the female genitalia (Vol 1): Part 2SN are only very rarely diagnosed, accounting for only 3% of all EST in the DKSM database (25). ESN have been observed to arise in women aged 23-86. TEbook Sarcoma of the female genitalia (Vol 1): Part 2
he majority of patients are premenopausal, with a median and mean age of 47 resp. 53 years. It can already be deduced from this age distribution that Giinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2with LG-ESS is 46 years, suggesting that, like ESN, the majority of such tumors arise in premenopausal women (25). These two types of neoplasm in fact share clear pathogenetic commonalities. On the one hand, the differences between the two in terms of their microscopic features are more gradual than Ebook Sarcoma of the female genitalia (Vol 1): Part 2 abrupt. Both l.G-ESS and ESN exhibit t(7;17) translocations and consecutive fusions of the JAZF1 and SUZ12 (previously: JJAZ1) genes as well as t(6;7Ebook Sarcoma of the female genitalia (Vol 1): Part 2
) translocations (JAZF1/SUZ12 gene fusion) (26). The t(7;17) (pl5:q21) translocation is regarded as an early genetic aberration in the development232 Giinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2ifferentiation, which allows the conclusion that such tumors originate from a progenitor cell (35), and could also serve to explain why ESN and LG-ESS so frequently arise within the myometrium. LG-ESS, ESN and CLM express common immunohistochemical markers (CD10, SMA, WT-1), another factor that supp Ebook Sarcoma of the female genitalia (Vol 1): Part 2orts the notion that sừomal and myogenic tumors might derive from the same precursor cells (48). What is rather conspicuous from an epidemiologic persEbook Sarcoma of the female genitalia (Vol 1): Part 2
pective is that ESN account for only a very small proportion of al) EST. Subtle analyses have revealed that, when ESN and LG-ESS are summed together, Giinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2SS and ESN is very difficult, this can essentially only be explained by the fact that a non-negligible number of ESN (for example such with limited infiltration or myogenic differentiation) are probably classified or diagnosed as LG-ESS just to be “on the safe side”. That could also help to explain Ebook Sarcoma of the female genitalia (Vol 1): Part 2why there is so much variation in terms of the propensity of such tumors to recur. The situation might actually be very similar to that of STUMP and IEbook Sarcoma of the female genitalia (Vol 1): Part 2
.MS.Endometrial stromal nodules are rare benign endometrial stromal tumors that predominantly arise in premenopausal women. They are regarded as a preGiinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2ss the surrounding myometrium, and potentially rhe endometrium as well. The tumor is thus well-circumscribed. ESN have a yellowish, tan, occasionally almost white color on the predominantly smooth, fleshy cut surface. Sometimes cystic-structures, necrosis and focal hemorrhages can be visible in the Ebook Sarcoma of the female genitalia (Vol 1): Part 2cut-open specimen. Nodular or polypoid growth into rhe cavuin uteri is not uncommon. ESN are usually considerably softer than LM. Their size can rangeEbook Sarcoma of the female genitalia (Vol 1): Part 2
from only a few mm to 22 cm, with a mean diameter of 5-7 cm. Multiple ESN can develop simultaneously.Microscopy also very frequently reveals that ESNGiinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2pear to resemble an actual capsule in some cases. The densely packed, diffusely growing small oval to spindle cells strongly resemble the endometrial stromal cells of the proliferative endometrium. The cells contain scant cytoplasm and usually have unclear margins. The oval to spindle, relatively un Ebook Sarcoma of the female genitalia (Vol 1): Part 2iform nuclei are practically void of pleomorphism, have unsuspicious nucleoli and exhibit a prominent coloration that often makes them appear deep bluEbook Sarcoma of the female genitalia (Vol 1): Part 2
e in histological specimens.The cells often focally whorl around the vessels (1). Virtually all ESN contain ample uniform vessels (15) reminiscent of Giinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2ferentiation in such cases.3.1 Endometrial stromal nodule ----- 233Densely cellular tumors are predominant (roughly 80 %), while 20 % are hypocel-lular with fibrous, hyalinized, myxoid or edematous sections (15). ESN with an abundance of epithelioid cells have also been described in the literature ( Ebook Sarcoma of the female genitalia (Vol 1): Part 233). Occasional areas consisting of foam cells within the tumor are another noticeable feature (31). There are no entirely cytologic criteria that canEbook Sarcoma of the female genitalia (Vol 1): Part 2
be drawn on to differentiate ESN from LG-ESS. Hyalinized areas, cystic degeneration and necroses occur in both of these types of tumors, and are thusGiinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2 (Fig. 3.1.1 (A)).Fig. 3.1.1 Histological findings of an endometrial stromal nodule and diagnostic differentiation from cellular leiomyoma; (A) a clear, sharp margin between the stromal nodule (top left) and rhe myometrium (bottom right) constitutes an important characteristic feature, the stromal c Ebook Sarcoma of the female genitalia (Vol 1): Part 2ells with scant cytoplasm morphologically correspond to those of low-grade endometrial stromal sarcoma; (B) cellular leiomyoma can have a very similarEbook Sarcoma of the female genitalia (Vol 1): Part 2
appearance and needs to be ruled out via immunohistochemical testing.The tumor margins can also be slightly irregular or poorly defined as a result oGiinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2ted no further than 3 mm away from the tumor does not serve to exclude such cases from being diagnosed as ESN. Tumors in such cases are referred to as ESN or EST with limited infiltration (15, 49, 51) or LG-ESS with limited infiltration (1). VI and LV1 are absent by definition. Tumors in which the i Ebook Sarcoma of the female genitalia (Vol 1): Part 2nfiltrations or satellite masses extend beyond 3 mm or in which there is VI will be LG-ESS. In some cases, tumors with focal margin irregularities thaEbook Sarcoma of the female genitalia (Vol 1): Part 2
t extend up to 9 mm beyond the main border of the tumor still do not have to be classified as LG-ESS (15). Such infiltrations usually only occur focalGiinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2ists, and constitute a substantial reason why ESN and LG-ESS share such overlap in a diagnostic “gray area”.2343 Endometrial stromal tumorsSmooth muscle differentiations are far from the exception in ESN, and are present in up to 50% of cases (15). At times, the smooth muscle cells can also appear t Ebook Sarcoma of the female genitalia (Vol 1): Part 2o resemble epithelioid cells. Tumors in such cases are referred to as ESN with smooth muscle differentiation, regardless of the extent of the smooth mEbook Sarcoma of the female genitalia (Vol 1): Part 2
uscle component (32). In the past, tumors in which the smooth muscle tissue accounted for >30% were termed stromal myoma. Today, these tumors constituGiinter Kohler, Katja Evert, Marek Zygmunt and Matthias Evert3 Endometrial stromal tumors - endometrial stromal nodule, endometrial stromal tumor with Ebook Sarcoma of the female genitalia (Vol 1): Part 2e “interlocked” with stromal cells in a cog- or phalanx-like fashion, a situation that can be misinterpreted as myometrial infiltration by stromal cells and thus result in a misdiagnosis as LG-ESS. Larger areas of smooth muscle differentiation can thus cause differential diagnostic problems, especia Ebook Sarcoma of the female genitalia (Vol 1): Part 2lly in curettage specimens (see below). Besides fibrous elements, 24 % of rumors also contain focal visible degrees of sex cord-like differentiation aEbook Sarcoma of the female genitalia (Vol 1): Part 2
rranged in cords/bundles in a glandular epithelioid structure (15). These tumors are further subdivided into two subtypes along the lines of the extenGọi ngay
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