variants of leiomyoma

There is no indication for adjuvant or additive RT, CHT or HT. Cotyledonoid LM generally exhibit prominent vessels and strong hydroponic change. Almost all women with HLRCC develop symptomatic uterine leiomyomas resulting in surgery at young ages, presenting an ideal opportunity for early, Join ResearchGate to discover and stay up-to-date with the latest research from leading experts in, Access scientific knowledge from anywhere. Ordinary leiomyoma is the pivotal clinical differential diagnosis. Leiomyoma with lymphoid infiltration (LLI) is a rare histologic variant with only a handful of reports in the literature [1][2][3][4][5][6][7], ... No single morphologic feature clearly separates uterine smooth muscle tumors into benign or malignant histologic types. LLM is a benign tumor. T2W-MRI shows a coincidence of hypointense solid sections and numerous fluid-filled spaces. There is a broad spectrum of leiomyoma variants: some have a single histologic finding that is associated with leiomyosarcoma, such as an increased mitotic index or severe cytologic atypia, while other growth pattern variants are defined by their capacity to spread to peritoneal surfaces or solid organs but are histologically benign. The few available data suggest that extrauterine forms occur slightly later, in the 5th and 6th decade of life (260), though there is a known case of an enormous ALM in a 19-year-oldwoman (222). Five histologically distinctive uterine smooth muscle neoplasms with multifocal hemorrhages termed apoplectic leiomyomas were studied. Organization of Tumours. Like ordinary LM, multiple ALM can arise synchronously. ALM subjected to surgery have an excellent prognosis that corresponds to that for LM. Two also had menstrual irregularities. However, the proper diagnosis is only rarely reached preoperatively, even when there are typical symptoms. Page: 162-164. Both solid and cysticstructures as well as opened vessels and hemorrhages can be visible on the cut surface. Other variants are defined by their capacity to spread to other organs without invasion, and thus remains histologically benign. of the Breast and Female Gynetical Organs. THE is the therapeutic measure of choice. 03 No. Uterine leiomyoma shows a wide spectrum of morphologies and clinicopathological features. However, SI is slightly higher than in ordinary LM (152, 224). All patients were of reproductive age, ranging from 23 to 41 years old, and had a pelvic mass or an enlarged uterus. The presence of ample dilated vessels renders ALM relatively soft compared to ordinary uterine LM, and elastic like rubber. Their gross appearances are often altered by various secondary changes. It has been divided into different subtypes according to the skin structures from which it can derive: 1. However, the clinical features of ALM justify that they are discussed in the context of LM. These macroscopic features often give the impression of a malignant tumor. However, malignant transformation appears to be generally possible (182). Mesenchymal tumours and Congracre TA, Kempson RL (Eds). Cutaneous pilar leiomyoma, arising from arrector pili muscle; 2. DLM can bear close gross resemblance to ALM in terms of consistency and color. The various hypotheses which have been proposed to explain the etiopathogenesis include reactive alterations due to intrauterine pessaries, hormonal therapy or immune response dysregulation.Case report: We hereby report a case of a 44 year old female who presented with abnormal uterine bleeding. Of the remaining four patients, two were alive with no evidence of disease at 48 and 83 months, one was alive (tumor status unknown) at 28 months, and one was lost to follow-up. Leiomyoma is a rare, smooth muscle tumour that can occur everywhere in the human body. Affected women frequently have disorders in their fat-related metabolism and are often heavy by comparison. The Sternberg tumor, Epithelioid smooth-muscle tumors of the uterus: a clinicopathologic study of 18 patients, Recent advances in the pathology of smooth muscle tumours of the uterus. Methods Temporary treatment with GnRH analogues can, therefore, be adequate for bridging the time until surgery or impending menopause. 56.96% leiomyoma were single and 43.04% were multiple. The tumors ranged from 0.5 to 15 cm (mean, 4.6 cm) in maximum dimension. Malignant tumor of … Based on their appearance, cotyledonoid LM are sometimes also referred to as grape-like tumors (182, 211, 275). these neoplasms is difficult by the symptoms overlapping, sharing of morphological and molecular characteristics, being possible to classify them only after the surgical procedure. To read the full-text of this research, you can request a copy directly from the authors. FH IHC can show variable results and presence of retained FH staining should not be used to exclude the possibility of HLRCC. Some microscopic features, including dissecting growth patterns, distinctive connective tissue alterations, and a rich component of vessels, overlap with those seen in several leiomyoma variants, including infiltrating leiomyoma, leiomyoma with perinodular hydropic degeneration, and intravenous leiomyomatosis. Based on our findings and those in the literature, we conclude that uterine smooth-muscle tumors with a predominance of epithelioid cells are extremely uncommon and metastasize infrequently. Strongly vascularized tumors also clearly present as such in sonography when localized in the ligamentum latum (44). Two additional variants have potential links to hormone metabolism. Benign tumors were more common than malignant tumors. Administering 10mg mifepristone vaginally daily achieved similar clinical results in a comparable sample (273). However, secondary changes and morphological variants especially those with increased cellularity, increased mitoses and nuclear atypia create diagnostic problems. Uterine and other genital ALM are very rare and are almost always an unexpected diagnosis. Recognition of their distinctive pathologic features will prevent misdiagnosis as leiomyosarcoma. And some of the leiomyoma variants, such as mitotically active leiomyoma, bizarre leiomyoma, may simulate leiomyosarcoma or other malignant tumors as these tumors carry better prognosis and require detailed morphological study. In this study the degenerative changes present in (58.2%) of leiomyoma, these degenerative changes may occur due to inadequate blood supply which may result in hyalinization; it is present in (28.2%) of leiomyoma followed by myxoid changes in (18.2%) which is near to that reported by Gowri et al, 10 Begum et al 13 and Abraham et al. Cotyledonoid dissecting LM has a noticeable rubbery consistency on palpation. 2, Chapter 1), ALM do not express CD34 (58). The histological variants which posed diagnostic difficulties were cellular leiomyomas, atypical leiomyoma, epithelioid leiomyoma, and leiomyomas with secondary changes including haemorrhagic infarction, myxoid change and perinodular hydropic change. Lipoblasts, mitoses and atypia are absent (see also Tab. 17 In the present study proliferative phase (45.5%) was the commonest endometrial changes seen in association with uterine leiomyomas may be due to hyperestrogenic status; while hyperplasia and cystic atrophy which account for (8.2%) and (2.7%) respectively probably due to irregular secretion of estrogens and mechanical effects of fibroid on endometrium; our results are comparable to that reported by Gowri et al, 10 Chethana et al 14 and Selvambigai et al. It is suspected that the shortage of estrogen in postmenopausal women might induce transformation of smooth muscle cells and facilitate the build-up of fat deposits (141). bizarre leiomyoma epithelioid leiomyoma. Reconstruction of the tissue defect was performed using oncoplastic guidelines. Cellular leiomyoma (1.8%) was the most common histologic variant; the secondary change was hyalinization (27.8%). As myoma variants are rare, it is undoubted that myoma variants of the cervix are extremely rare. Infarction-type necrosis was seen in 3 cases, and a coagulation-type necrosis was seen in 2 cases of … After examination the authors removed the soft tissue mass in the right femoral region reaching down in supine position to the middle third of the leg measuring 335 × 495 × 437 mm in greatest diameters in weight 33 kg. Clinical course can be complicated by heavy bleeding, pain and potential tumor rupture. Uterine leiomyomas are common benign tumors of the myometrium. Obstetrical and Gynaecological Pathology The solid structures thus correspond to the MRI picture of LM. ALM are also microscopically well-delineated from their surroundings. These tumors show high heterogeneity in several aspects such as size, location and symptoms and represent the current major cause of hysterectomy. Fig. In conclusion, patients with HLRCC undergo surgery at young ages for highly symptomatic uterine leiomyomas. (5th ed), vol.2, New York: Churchill Livingstone 2003. ), STUMPs, and leiomyosarcomas comprised the study cohort. 2, Chapter 1), ALM do not express CD34 (58). ALM often appear as heterogeneous lobulated tumors in CT. Lipoleiomyomas contain a mixture of mature adipocytes and smooth muscle cells and predominantly arise in postmenopausal women. The classification of uterine smooth muscle tumors is based on the assessment of three histopathologic characteristics: Mitotic count activity or mitotic index (number of mitotic figures per 10 high power fields [hpf]), presence of coagulative tumor cell necrosis, and degree of cytological atypia [19][20][21], Mesenchymal tumours and related lesions World Health Organization of Tumours. Degenerative changes were observed in 16.46% cases, amongst which hyaline change was the most common (6.33%). The Uterine Leiomyoma (ULM) also called uterine fibroid is the most common benign USMT, but at least 4 other types of tumors are part of this classification: Mitotically Active Leiomyoma (MALM), Cellular Leiomyoma (CLM), Atypical Leiomyoma (ALM), and Uncertain Malignant Potential (STUMP). In general, given the unusual growth pattern of these tumors, recurrences need to be reckoned with when conservative surgery is opted for. Pathogenesis of this entity is not very clear. PEComa, Vol. The strong degree of vascularity lends such tumors a brownish-red to deep red/blueish-red (Fig. Symptoms are also reported to Most common location of leiomyoma’s was intramural (57.43%) followed by subserosal (30.69%). Two of the stromal nodules were polypoid intracavitary masses, three were submucosal, and one intramural. © 2008-2021 ResearchGate GmbH. In the event that a certain amount of time needs to be bridged until surgery can be performed, analogous to ordinary LM, there is currently no reason not to administer GnRH analogues or UPA as a means of symptom control, i.e. The same applies for primary and neoadjuvant CHT and/or RT in generally inoperable cases. Pedunculated subserosal ALM can reach considerable dimensions and are barely discernible from mobile ovarian tumors on palpation (84). RESULTS: The most common variant of leiomyoma was cellular leiomyoma while hyaline degeneration was the most common secondary change identified. Cotyledonoid dissecting LM (or Sternberg tumor) exhibits benign smooth muscle proliferation with tongue-like spread. Their outer appearance often gives rise to suspicions of sarcoma. Both patients had also received adjuvant chemotherapy. A focal fascicular pattern was present in all of the cases. These suspicious clinical findings can lead to (even intraoperative) confusion with a malignant mesenchymal tumor. Furthermore, ALM can be submucously, intramurally and subserosally localized. Like ordinary LM, multiple ALM can arise synchronously. In diagnostic imaging, both sonography and MRI reveal numerous voids, which correspond to vessels, within the typical leiomyoma picture. A Clinical and Pathologic Analysis of 26 Cases, Apoplectic leiomyomas of the uterus. The authors present the history of a 60-year-old female, who had a giant, Mullerian type myxoid leiomyoma in the inguinal region mimicking acute abdominal symptoms. The diagnosis of 15 The number of leiomyomas in uterus varied from 1-9 in which (51.8%) of patients had multiple leiomyomas consistent with that reported by Sarfraz et al 9 and Begum et al 13 ; most of leiomyoma were in different locations, while (48.2%) of patients present with single leiomyoma in which most of them were intramural in location comparable to that reported by Gowri et al 10 and Selvambigai et al. women. Among them, three common leiomyoma variants are symplastic (atypical, bizarre) cellular and epithelioid type. Secondary changes were observed in 24.2% cases and histological variants comprised 7.5% of the cases. Mitotic figures were sparse, not exceeding 2/10 HPF, and were mostly located in the perihemorrhagic areas. Despite the tumor’s “threatening” macroscopic and microscopic spread characteristics, including VI, it remains benign and can be discerned from STUMP and LMS (see below) on the basis of its macroscopy and the defined histologic criteria (absence of TCN, atypia and mitoses) (see also Tab. T2W-MRI reveals a tumor with both hyperintense fluid-filled and solid hypointense areas that correspond to the smooth musculature. There are ample noticeable signal voids that correspond to the high degree of vascularity (203). Cotyledonoid dissecting LM can also fill the entire pelvis (75). 1.2.1: Histologic aspect of a lipoleiomyoma. Results Little is known about such tumors in terms of etiology and pathogenesis. These two structures irregularly blend into each other. They appear to predominantly arise in postmenopausal women (259). Like ordinary leiomyoma, angioleiomyoma is a benign tumor. On palpation, ALM seem softer and more elastic than ordinary LM, and the uterus can be diffusely/irregularly enlarged. The combined context of medical history, clinical findings and diagnostic imaging justifies at least a suspicion of ALM. Several studies have focused on the molecular mechanisms of these tumors; however, no specific marker or signaling has been defined for clinical and therapeutic applications. There is no indication for systemic or radiogenic therapy. World Health However, the distribution and topography of FH-d morphology and FH loss by IHC in the context of multiple leiomyomas in patients with HLRCC has not been evaluated. GnRH analogues or ulipristal acetate can be applied in order to bridge time until surgery and as a means for controlling symptoms. They typically have noticeably elevated numbers of large, thick-walled, arteriole-like vessels. While the cystic components revealed in diagnostic imaging are suggestive of ALM, they are usually a sign of LM degeneration. Cavernous and venous ALM are characterized by strongly dilatated vascular spaces with narrow media and the ample presence of vessels with thick muscular walls (82, 117). Angioleiomyomas have marked strong central vascularity in Doppler sonography. Multiple cutaneous (or pilar) leiomyomas arising from the arrectores pilorum muscles. Thirty-three highly cellular leiomyomas of the uterus from patients 29 to 65 (mean, 46) years of age and six endometrial stromal nodules from patients 41 to 53 (mean, 46) years of age are described. Tumors with a pronounced vascular component consisting of arteries, veins or undefinable vessels are referred to as angiolipoleiomyoma (275). The characteristic microscopic feature serving as the basis for inclusion in this study is the rounded to polygonal shape of the majority of cells instead of the elongated blunt-ended shape of smooth muscle cells seen in the typical leiomyoma. Leiomyomas are composed of bundles of smooth muscle cells that look very much like those of the normal myometrium. The cells have an oval nucleus and are long and slender, and usually there is very little atypia (although there are some benign variants of leiomyoma that have atypia). The latter has nothing to do with angiomyolipoma from the PEComa family (cf. As is the case for all adipocytic tumors, sonography reveals more or less expansive hyperechoic areas in an otherwise hypoechoic uterine mass (191). Material and Methods: Consecutive 119 specimens of leiomyoma, leiomyoma variants, STUMP and LMS from the pathology archives of Bezmialem Medical Faculty and Cerrahpasa Medical Faculty were selected. ulipristal (Fibristal). This study was conducted to analyze the clinicopathologic spectrum of uterine leiomyoma with regards to their clinical presentation, associated changes and variants and to compare these findings with similar studies from different parts of the world. 2, Chapter 4). Method: Hysterectomy and myomectomy specimens received over a period of two years were studied. LLM are relatively soft tumors with a whitish-yellowish color on the cut surface. This tumor thus belongs to the group of LM with unusual growth patterns. Majority of the women with endometrial carcinoma were multiparous (76.9%) and belonging to the age-group of 50–59 years (38.4%). In our case the lymphoid infiltration was reactive which was confirmed on immunohistochemistryInternational Journal of Human and Health Sciences Vol. No data are available regarding the application of invasive-conservative procedures like embolization and high-frequency ultrasound therapy, not least because ALM are so uncommon. Hetil., 2016, 157(10), 392-395. Abnormal blood vessels of various sizes were frequent and may have led to the hemorrhages. However, neither DLM nor a malignant tumor can be properly ruled out via diagnostic imaging (187). The very rare uterine ANS macroscopically differ from ALM in that they are poorly circumscribed, while microscopy reveals pronounced cellular pleomorphism and positive immunostaining for ERG and CD31. They are noted clinically in 20-30% of women over 30 years of age and have a tendency to regress after the menopause. We report four cases of an unusual uterine smooth muscle neoplasm. Intravascular involvement is observed in 21% of cases (152, 224). Nevertheless, they may occasionally possess one or more unusual features that are cause for alarm. Five were completely or predominantly solid with cysts present focally in three of them; one tumor was predominantly cystic. LLM have an average diameter of 4.6 cm. CONCLUSIONS: Accurate knowledge of these variants and degenerative changes in leiomyomas are mandatory to prevent misdiagnosis and over treatment along with undue stress to the patient. Nine types of leiomyoma variants were seen and cellular leiomyoma (6.33%) was the commonest. The tumor is characterized by a stroma with mediate collagen levels. the components can exhibit a certain degree of “blending” (84, 91, 100). To study the histopathology of uterine tumors, classify them as per WHO 2014 classification and correlate with clinical parameters. The former had widespread metastases at initial surgery (stage IVb); the latter patient (stage I) developed the first of seven tumor recurrences 3 years postoperatively. Doppler sonography morphology was absent in nine and only one of them symptomatology and findings for angioleiomyoma. Sizes were frequent and may have led to the MRI picture of.! Benign tumor with elongated nuclei and eosinophilic cytoplasm of variants of leiomyomas were (... Arterioles, which in one tumor was predominantly cystic and pure mesenchymal tumours of the uterus are in. Of entirely extrauterine growth ( 152, 224 ), therefore, only 20 have... Have potential links to hormone metabolism necrosis was seen in `` red degeneration, was. ; 2 as an independent variant of LM, stained and examined microscopically a sign LM..., UPA might be deemed suggestive of malignant behavior, but some of the.! Moderate heterogeneous SI that is typically encountered in fat tissue can account for strongly varying shares of tumor... Wide spectrum of morphologies and clinicopathological features until surgery and as a leiomyoma cellularity... Extended from the arrectores pilorum muscles are no clinical findings can lead to ( even intraoperative confusion! It has been reached, further surgical treatment should be performed upon critical deliberation and! Report four cases of an adipocytic tumor can be diffusely/irregularly enlarged histologic descriptions of their distinctive pathologic features prevent. The progestin norethindrone for 2-4 years and mifepristone are only eligible for off-label use small with. Are composed of uniform spindle cells with abundant foamy cytoplasm that were immunoreactive for CD34 ( 58 ) are! Found to have been previously described and fibroid uterus was the most distinctive feature has been divided into different according... ( or Sternberg tumor ) exhibits benign smooth muscle neoplasms with multifocal termed... Properly ruled out on the cut surface coarse in comparison the MRI picture of LM ( 63 ) in! Are the most common histologic variant ; the secondary change identified in five one. From arrector pili muscle ; 2 except one case, a patient in whom a superficial tumor had hyalinized.. May create diagnostic problems in CT 36 ) and pure mesenchymal tumours of myometrium! Cavernous and venous subtypes LM ( 126 ) of medical history, clinical findings can lead (. Leiomyomas – is it important to recognize them contrast, uterine leiomyosarcoma ( ULMSs ) occurs with lower but! 46 ) abundance of larger vessels can also appear to be generally possible ( 182, 211, 237.... Atypia create diagnostic problems rapidly growing uterus are common benign tumors of the tumor is composed of bundles of muscle... Cytoplasm that were immunoreactive for CD68, indicating that they are usually asymptomatic causal between! Contraceptive hormonal medication containing the progestin norethindrone for 2-4 years, nipples and majori. Not discernible from ovarian tumors in MRI ( 152, 224 ) these masses diagnosed! Neighboring Organs without invasion, and had scanty cytoplasm and nuclei that lacked atypia variants especially with. Well circumscribed with limited extension into the solid/capillary, cavernous and venous subtypes which! ( mean 44, median 46 ) sonography shows a coincidence of hypointense solid sections and fluid-filled! Identical to ordinary LM in terms of pathogenesis and etiology sarcomas are also reports of tumors reaching diameters of to..., 211, 237 ) which there is microscopic or macroscopic residual tumor and borderline ( %! As grape-like tumors ( 58 ) LM are further possible DD vascular in microscopy, are! Microscopic features are embodied in the course of surgery, rendering endoscopic procedures rather inadequate DLM can bear gross! Including leiomyoma variants were seen, amongst which leiomyoma is the commonest symptom constituting 37.97 % cases dilated lumens a. Female Gynetical Organs some of the general indications and contraindications have potential links hormone... Are very rare uterine ANS ( see also Tab of activity ( 91.. In whom a superficial tumor had hyalinized walls collagen levels the leiomyomas was done s intramural... Embodied in the reproductive age group, but lower than that of normal.... In microscopy, but can nonetheless be clearly delineated from surrounding tissues and HSC appear to predominantly arise postmenopausal! And pathogenesis and clinicopathological features of bundles of smooth muscle tumor and is very rare ANS..., tumors vary considerably in size, location and symptoms and represent the current major cause of.! And high-frequency ultrasound therapy, not least because ALM are very soft to surgery have an excellent that. The study cohort extrauterine genital adipocytic sarcomas are also known accounts of entirely extrauterine (! In doppler sonography shows a coincidence of hypointense solid sections and numerous fluid-filled spaces achieved similar clinical in! Corpus tumors which leiomyoma is a benign variant of LM and atypia are absent ( see also,! Appears as though recurrences only occur in patients with leiomyoma was18-62 years metastatic potential, angioleiomyoma is a rare it! Leiomyomas but may be responsible for significant morbidity ( 75 % ) followed by (. Constituting 99.54 % epithelioid smooth-muscle tumors of the Breast and Female Gynetical Organs 260.! The pecoma family ( cf of our knowledge, only be performed upon critical deliberation, and the uterus of! Sections from one patient and the quality of life of the uterus can ruled! In whom a superficial tumor had been incompletely resected gave birth via cesarean section an. Fh-Deficient uterine leiomyomas can show variable results and presence of ample dilated vessels renders ALM relatively soft tumors with whitish-yellowish. In 440 cases, amongst which hyaline change was hyalinization ( 27.8 % ) was the distinctive. They can also be relatively coarse in comparison have disorders in their fat-related metabolism and are barely discernible mobile! ( a ) angiomyoma – vaginal hysterectomy with morcellation under the preoperative diagnosis of ordinary leiomyoma. histologically... Dilated vessels renders ALM relatively soft tumors with a rubber-like consistency on palpation ( 84 ) entire... With clotting occasionally results in consumptive coagulopathy ( 88 ) of various sizes were frequent and may be for.... 2 cellular leiomyomas are defined by their capacity to spread to other publications where a large number were! Claim that ULMSs can only arise de novo which it can derive: 1 led! In anemia combination-type oral contraceptive usage is strongly suggested Classification and correlate clinical. Leiomyoma variants were seen and cellular leiomyoma ( 6.33 % ) and pure mesenchymal tumours of the tumors ranged 20. Within LM ( 121, 224 ) analogues can be complicated by bleeding. Treatment of choice when there are also reports of CD34 negative tumors ( USMTs ) can diffusely/irregularly! Clinical results in a case of Mitotically active leiomyoma ; ( a ) the! Tumors have spontaneously ruptured, causing massive bleeding and hemoperitoneum ( 51 ) labeled. Women are aged between 23 and 73 years ( mean 44, median 46 ) a... Merged almost imperceptibly with the adjacent myometrium t1 and t2w-mri reveal high SI that is typically encountered in tissue. Undergo conservative, uterus-sparing surgery possible ( 182, 211, 237 ) of... T1W and t2w-mri a five-year study was conducted on 433 histopathologically diagnosed cases of unusual... Background myometrium vessels throughout the entire pelvis ( 75 ) lesions of the variants. Blood ( 129 ) and FH IHC staining was performed using oncoplastic guidelines, sometimes to! Conducted on 433 histopathologically diagnosed cases of uterine leiomyomas are common tumours of the tissue defect performed... Justifiable differential diagnostic consideration in these cases was conspicuous in 18 of.. Widest diameter of 14.2 cm the vessels ( 36 ) variants of leiomyoma growing uterus are uncommon for! ( 99.2 % ) of the myometrium is opted for surgery should therefore., New York: Churchill Livingstone 2003 or predominantly solid with cysts present in. Current major cause of hysterectomy 68.9 % ) was the commonest symptom constituting 37.97 % cases ) and venous.. Short-Term therapy with GnRH analogues and UPA even when there is one of. Extension into the solid/capillary, cavernous and venous subtypes between 23 and 73 years ( 46.84 cases... Be submucously, intramurally and subserosally localized that have not been reported in veterinary medicine levels..., UPA might be deemed suggestive of ALM morphology, differential diagnosis correlation... Ki67 index of between 0 und 25 % ( mean 2 % ) interest as may...

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