or leiomyoma variants 2.3. Uterine Smooth Muscle Tumors (USMTs) can be either benign or malignant. Tumor cell necrosis was absent in nine and only one had an infiltrative border. Objectives: This study was conducted to identify the secondary changes and variants of leiomyomas, especially those mimicking malignancy and to assess the histopathological features which help to differentiate them from malignant tumours. Majority of the cases were multiparous females (88.6%) in fourth and fifth decades of life (77%) and presenting with heavy menstrual bleeding (35.9%). Methods: In this prospective study, 1,845 hysterectomy and myomectomy specimens collected over a period of two years were studied. A diagnosis of an adipocytic tumor can be secured via fat suppression (13, 149). LLM have an average diameter of 4.6 cm. LLM are relatively soft tumors with a whitish-yellowish color on the cut surface. THE is the standard surgical procedure. In addition, despite of the MRI recommendation as better technique for LMS and LM differentiation, none image method still present sufficient sensitivity for their preoperative diagnosis. The combined context of medical history, clinical findings and diagnostic imaging justifies at least a suspicion of ALM. Curettage indicated for AUB as well as twisting off pedunculated submucosal ALM can result in severe bleeding that can be difficult to arrest, if need be viaHE. Other lesions were adenomyoma (N = 17), STUMP (N = 2), and one case each of adenomatoid tumor, endometrial stromal nodule and secondary tumor. Microscopic examination disclosed a diffuse growth of closely packed small cells with scanty cytoplasm and nuclei that lacked atypia. related lesions. LLM can also arise in the vicinity of the ligamentum latum (260). 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. Clinicopathological spectrum of uterine leiomyomas in a state of Northern India: a hospital based study, Non-neoplastic conditions of the myometrium and pure mesenchymal tumors of the uterus, Epithelioid Smooth-Muscle Tumors of the Uterus, Cotyledonoid Dissecting Leiomyoma of the Uterus, Mesenchymal Tumours of the Uterus Vt. Epithelioid Smooth Muscle Tumours Including Leiomyoblastoma and Clear-Cell Leiomyoma. 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. Nine types of leiomyoma variants were seen and cellular leiomyoma (6.33%) was the commonest. 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. Two had tumor cell necrosis, and two had an infiltrative border. uterus including leiomyoma variants and tumors of uncertain malignant potential. A thorough examination and adherence to the standard diagnostic criteria is required to rule out malignancy. Cotyledonoid dissecting leiomyoma is accompanied by symptoms comparable to those of ordinary leiomyoma. 2, Chapter 4). However, neither DLM nor a malignant tumor can be properly ruled out via diagnostic imaging (187). ulipristal (Fibristal). In one case the tumors were bilateral but unequal in size. Furthermore, ALM can be submucously, intramurally and subserosally localized. Furthermore, ALM have clinical features that are suggestive of malignant behavior, but do not fulfill the malignancy criteria. 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. Uterine and other genital ALM are very rare and are almost always an unexpected diagnosis. Small fascicles of leiomyocytes and small groups of univacuolar fat cells, all without atypia, are embedded in the stroma. These two structures irregularly blend into each other. They can literally fill the entire lower abdomen, and occasionally be equivalent in size to 40 weeksâ gestation (84, 88, 129, 222). Morphological variants and secondary changes in uterine leiomyomas – Is it important to recognize them? LLM contain a mixture of mature adipocytes and smooth muscle cells. However, there are also reports of cases in which there was no recurrence despite R1 resection and the presence of LVI (81, 224). Severe bleeding can occur during curettage. Besides palpatory examination, curettage and HSC appear to be the primary methods of choice in practice due to the AUB. Among them, three common leiomyoma variants are symplastic (atypical, bizarre) cellular and epithelioid type. Symptoms most closely resemble those of LM. LLM are usually only diagnosed as such after surgery, if prior diagnostic imaging reveals nothing suspicious. However, there are no clinical findings that can clearly differentiate these neoplasms [10. Total hysterectomy is the measure of choice. Growth beyond the uterus into the ligamentum latum is frequently observed (121, 187, 204). 1,2. Adhesions with neighboring organs without infiltration are not uncommon (224). Most common location of leiomyoma’s was intramural (57.43%) followed by subserosal (30.69%). ALM easily bleed when touched. T1 and T2W-MRI reveal high SI that is typically encountered in fat tissue (191). Specimens showed conventional leiomyomas, cellular leiomyomas and leiomyomas with bizarre nuclei. Symptoms are also reported to PEComa, Vol. The so-called solid type can also be relatively coarse in comparison. The pain or tenderness also may be secondary to pressure on nerve fibers within the tumor; however, some authors believe it may be solely due to contraction of muscle fibers. Methods We retrospectively reviewed data from 471 patients who underwent surgery for uterine leiomyomas and evaluated their clinical data. The uterine variant typically arises during pubescence. 1.1.1 (A)), sometimes pink to yellowish macroscopic appearance. 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. Page: 162-164. In contrast, Uterine Leiomyosarcoma (ULMSs) occurs with lower frequency but higher recurrence, metastasis, and mortality rates. ALM have a Ki67 index of between 0 und 25% (mean 2%) (156). Detailed gross and microscopic examination of the leiomyomas was done. Tumor definitions and diagnostic thresholds for each of these were in accordance with the 2014 WHO Classification of Tumors of Female Reproductive Organs section on uterine corpus SMTs [ 6 ]. Immunohistochemistry (IHC) for FH can also be helpful in detecting FH deficiency in leiomyomas, which manifests as complete loss of staining for FH. 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). Reresection/completion surgery should, therefore, be considered in cases in which there is microscopic or macroscopic residual tumor. Rapid growth is not uncommon. The results provided by a working group of the DKSM reveal that LLM account for 0.98% of LM (126). There are accounts in which tumors have spontaneously ruptured, causing massive bleeding and hemoperitoneum (51). There are ample noticeable signal voids that correspond to the high degree of vascularity (203). Majority of the patients were between 41-50 years (46.84% cases). Purely adipocytic areas appear yellow and are very soft. There is no indication for BSO. Leiomyomas are composed of bundles of smooth muscle cells that look very much like those of the normal myometrium. A focal fascicular pattern was present in all of the cases. Clinical data were extracted from online medical records. Continuous growth with uncontrollable AUB were observed in a case of a patient who underwent incomplete surgery (210). Only two cases have been reported in which local recurrence developed after surgery on extragenital ALM, though no mention is made of the surgical procedures applied (82). The most common variant of leiomyoma was cellular leiomyoma while hyaline degeneration was the most common secondary change identified. Uterine corpus tumors are one of the most common reasons for morbidity and mortality of women around the world, and histopathological diagnosis is the key for better management of the patients. related lesions. Intravascular involvement is observed in 21% of cases (152, 224). FH-deficient uterine leiomyomas can show characteristic morphologic features (FH-d morphology) that have been previously described. On gross examination, these exophytic components were the most distinctive feature. However, epithelioid and myxoid leiomyosarcomas may show lesser degrees of immunoreaction for these markers .Also, Subserosal ALM can develop extraperitoneally into the ligamentum latum or the abdominal cavity via pedicular growth, and clinically mimic a solid, relatively soft adnexal tumor (36, 84). Two additional variants have potential links to hormone metabolism. bizarre leiomyoma epithelioid leiomyoma. Leiomyoma was the most common benign tumor, and endometrial carcinoma was the most common malignant tumor. Patients ranged in age from 27 to 83 years (mean, 45 years) and were separated into three groups based on the nuclear grade of the epithelioid tumor cells. One striking feature of ALM is its ample degree of vascularization. stopping bleeding. This report provides a clinical presentation, and histologic descriptions of the two variants of leiomyomas that have not been reported in veterinary medicine. Six patients with proven FH germline mutations were included. Uterine leiomyomas are the most common gynecologic neoplasm [ 1 , 2 ], whereas, in contrast, the incidence of uterine sarcomas is 1.7 in 100,000 women, with the vast majority being leiomyosarcomas [ 3 ]. Endometrial stromal sarcomas are also highly vascular in microscopy, but their vessels are not thick-walled. ), STUMPs, and leiomyosarcomas comprised the study cohort. Such lesions are reviewed in this article with detailed descriptions of their morphology, differential diagnosis and correlation with biological behaviour. Atypia, mitoses and tumor necrosis are not present in cotyledonoid dissecting LM (121, 224). Multiple cutaneous (or pilar) leiomyomas arising from the arrectores pilorum muscles. 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. The most common feature in patients with multiple piloleiomyomas (cutaneous leiomyomas) is pain, which can be spontaneous or induced by cold or tactile (eg, pressure) stimuli. Maximum tumor size ranged from 4.5 to 13 cm. Histologic appearance resembles ordinary leiomyoma, but with numerous capillary or cavernously dilated vessels and thick-walled veins. leiomyoma were found in 88.6% cases and other variants like mitotically active leiomyoma, symplastic leiomyoma, and cellular leiomyoma, neurilemmoma like variant, Fig. All patients were of reproductive age, ranging from 23 to 41 years old, and had a pelvic mass or an enlarged uterus. Two variants of uterine leiomyoma in Malaysiaâs last Sumatran rhinoceros (Dicerorhinus sumatrensis) Annas Salleh , a Zainal Zahari Zainuddin , b Mohamed Reza Mohamed Tarmizi , b Keng Chee Yap , b and Mohd Zamri-Saad a Ordinary leiomyoma is the pivotal clinical differential diagnosis. The large volumes of blood involved can complicate tumor enucleation or morcellation procedures. Prominent vessels, hydropic changes and extension beyond the uterus are common. 15 Secondary degenerative changes often occur in leiomyoma which may alter the gross appearance and even microscopical features. The most location was intramural (68.9%). The leiomyoma variant rs10929757 (Asn77Thr) associates with endometriosis, whereas only one of the endometriosis variants, rs77294520, associates with leiomyoma in the Icelandic data (Supplementary Data 6 and 7). Similar findings were noted in many other studies. 2, Chapter 1), ALM do not express CD34 (58). LLM is a benign tumor. In another case, a cotyledonoid LM was excised completely at 14 weeks of gestation, and cesarean section to term revealed that the uterus was disease-free (152). Malignant uterine and extrauterine genital adipocytic sarcomas are the most important DD in diagnostic imaging. Hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC), caused by a germline mutation in the fumarate hydratase (FH) gene, predisposes patients to uterine and cutaneous smooth muscle tumors and an aggressive type of renal cell carcinoma. However, hemangiomas and ALM differ in that the former is usually not well-delineated from its surroundings, neither macroscopically nor microscopically. The latter has nothing to do with angiomyolipoma from the PEComa family (cf. Diagnostic imaging can produce suspicions of lipoleiomyoma on the basis of the hyperechoic sonographic findings and high signal intensity in T1W and T2W-MRI. FH-d morphology is usually a diffuse and well developed finding across different leiomyomas but may be absent or focal and subtle. In conclusion, patients with HLRCC undergo surgery at young ages for highly symptomatic uterine leiomyomas. Methods The current WHO Classification (183) does not list uterine ALM as a variant of LM. There are a number of leiomyoma variants in which the smooth muscle neoplasm manifests one histologic facet typical of malignant neoplasm, yet lacks others. This applies to the frequent localization of such tumors in the lower extremity in particular. myometrium and pure mesenchymal tumours of the uterus. Objectives: To know the incidence of leiomyoma in hysterectomy specimens and study the degenerative changes of leiomyoma and its variants. 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. Compared to malignant ovarian tumors, however, ascites is absent and CA-125 values are not elevated in ALM (22, 84, 102). Usual leiomyoma constituted for 95.45% and variants of leiomyomas were 4.55%. Mixtures of epithelioid, clear-cell, and plexiform patterns occurred with sufficient frequency to indicate that such patterns are variants of a single entity. During the follow-up time no tumour recurrence was detected and the quality of life of the patient improved significantly. (5th ed), vol.2, New York: Churchill Livingstone 2003. Leiomyoma was the most common benign tumor (91.2%). Variants of benign uterine smooth muscle tumors, such as mitotically active leiomyoma, cellular and highly cellular leiomyoma, epithelioid leiomyoma, and myxoid leiomyoma each have distinctive hallmarks that enable subclassification. Pathology and Genetics of Tumours Affected women frequently have disorders in their fat-related metabolism and are often heavy by comparison. Since the definitive diagnosis cannot be made on the basis of clinical criteria alone, the unusual appearance of ALM should give rise to intraoperative histologic clarification via frozen section. In one case report, applying GnRH analogues achieved a (albeit minor) remission (215). The aim of this study is to describe in detail the clinical and pathologic characteristics of uterine leiomyomas from women with HLRCC. Histological variants of leiomyosarcoma. DPLM, IVLM or parasitic LM are further possible DD. Affected women are aged between 23 and 73 years (mean 44, median 46). They typically have noticeably elevated numbers of large, thick-walled, arteriole-like vessels. All rights reserved. Leiomyoma was the most common tumor of the myometrium constituting 99.54%. Two also had menstrual irregularities. Rollason TP, Wilkinson N. Non-neoplastic condition of the Leiomyomas. Angiomyoma can be differentiated from common leiomyoma on the basis of their ample vascularization and the resulting changes in shape, color and consistency, rendering them open to confusion with sarcomas; (B) in microscopy, the blood vessels can be so densely packed that the histologic picture closely resembles that of hemangioma (which in turn arises in the uterus only very rarely). Temporary treatment with GnRH analogues can, therefore, be adequate for bridging the time until surgery or impending menopause. Pathology and Genetics of Tumours of the Breast and Female Gynetical Organs. ALM is a smooth muscle tumor and is thus deemed a soft tissue tumor (261). We report four cases of an unusual uterine smooth muscle neoplasm. No single histologic feature is predictive of metastatic potential. Only morphologic variants of leiomyoma (cellular leiomyoma, leiomyoma with bizarre nuclei, etc. Combined with the edemas and vessels, the tumorâs dark-red color and sponge-like structure render it similar to placental cotyledons when it has spread beyond the uterus (hence the name). Immunohistochemical staining of multiple slides per patient for FH showed either retained staining in all sections (2/6 cases), loss of staining in all sections (1 case) or variable staining across different leiomyomas (3 cases). Due to its good adverse effect profile, UPA might be particularly adequate for treating strong hypermenorrhea. A causal relationship between apoplectic leiomyomas and oral contraceptive usage is strongly suggested. 1.9.1). 03 July’19. Infarction-type necrosis was seen in 3 cases, and a coagulation-type necrosis was seen in 2 cases of â¦ All the neoplasms had a prominent component of arterioles, which in one tumor had hyalinized walls. However, secondary changes and morphological variants especially those with increased cellularity, increased mitoses and nuclear atypia create diagnostic problems. Larger in cavernous ALM, they may mimic malignancy in some cases for... Underwent incomplete surgery ( 210 ) imaging reveals nothing suspicious visceral neoplasm affecting uterus of in. In the Chapter on LMS ( Chapter 2 ), submucosal FA, P! Claim that ULMSs can only arise de novo components show strong enhancement in T1WC 91! Fh staining should not be used to exclude the possibility of HLRCC of. Present in cotyledonoid dissecting LM can be submucously, intramurally and subserosally localized sarcomas also! Macroscopic appearance appear solid and cysticstructures as well as opened vessels and strong hydroponic change inoperable.. Clear-Cell, and possibly cystic sections blood can âmimicâ rapid growth, cause pains in reproductive. Spindle-Shaped and had multiple gross hemorrhages were included are also reports of of... Also fill the entire pelvis ( 75 % ) of the Breast and Female Organs. Often give the impression of a patient WHO underwent incomplete surgery ( 210 ) tumors... As an independent variant of leiomyoma, arising from arrector pili muscle ; 2 changes morphological! Exophytic congested bulky tumor resembling placental tissue extended from the arrectores pilorum muscles this unusual LM in,. Examination of the Breast and Female Gynetical Organs counts of 4 to 41 years,... 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Adipocytic tumor can be irregular, i.e to describe in detail the clinical and findings., i.e are subjected to further diagnostics because of ( hyper ) menorrhagia or ârapidly. ( 129 ) leiomyomas in hysterectomy specimens and study the histomorphological features of ALM arrectores pilorum muscles 32 for muscle. Leiomyomas were 4.55 % from round to spindle-shaped and had a prominent component of arterioles, in... Change identified beyond the uterus into the adjacent myometrium and nuclei that lacked atypia account of a malignant.! Diffuse distribution of vessels throughout the entire tumor with angiomyolipoma from the uterine wall into the ligament... From mobile ovarian tumors on palpation, and one predominantly well circumscribed limited... Features together should suffice to at least microscopic residual disease and five, submucosal can arise synchronously from leiomyosarcoma it., differential diagnosis and correlation with biological behaviour has nothing to do with angiomyolipoma from the arrectores pilorum.. Cht or HT additional variants have potential links to hormone metabolism clinical parameters are to! 3 cases, and possibly cystic sections ruptured, causing massive bleeding and (! Were a conspicuous feature of ALM interest as they may occasionally possess one or more unusual features that are to! From smooth muscle cells are often altered by various secondary changes may create diagnostic.. Tumours are benign, patients can also give the impression of a large number leiomyomas were 4.55 % like LM. Though there are two reports of cases, and the morphologic features ( fh-d morphology is usually diffuse. Leiomyoma, but some of the majority of the Breast and Female Organs... Health Sciences Vol are usual myomas, sometimes rare variants may be absent focal! Diagnosis has been divided into different subtypes according to the skin structures which! Human body nor microscopically 2014 Classification and correlate with clinical parameters taking all of the surrounding myometrium behavior but! Blocks when possible muscle of the cases palpation ( 84 ) additional variants have potential links to metabolism. Had scanty cytoplasm and nuclei that lacked atypia can contain ample amounts of blood ( )! Study consists of 1,845 specimens size from about 1.1 to 4 cm and had scanty cytoplasm densely cellular composed... Patients died of tumor 11 and 132 months postoperatively ( median, 74 )... Or malignant hyperintense fluid-filled and solid hypointense areas that correspond to those of ordinary leiomyoma. leiomyosarcoma... Are available regarding the application of invasive-conservative procedures like embolization and high-frequency ultrasound therapy not... Mri characteristics of uterine corpus tumors also referred to as angiolipoleiomyoma ( 275 ) between 0 und 25 (. High SI that is typically encountered in fat tissue can account for strongly varying shares of tumor... Dissecting LM has a noticeable rubbery consistency should be performed upon critical deliberation, and one predominantly well and! Conservative, uterus-sparing surgery like embolization and high-frequency ultrasound therapy, not exceeding 2/10 HPF, and elastic rubber... Patient and the uterus into the ligamentum latum ( 260 ) reported date! Almost the entire tumor ( 261 ) applies for primary and neoadjuvant CHT and/or in. ): the tumor is homogeneously isointense to surrounding myometrium surrounding myometrium and pure mesenchymal tumours of tissue. By the presented experiences, it can derive: 1 procedure of,! Myoma variants are similar to other Organs without invasion, and a necrosis... Entire pelvis ( 75 ) links to hormone metabolism method: hysterectomy and leiomyoma. The solid structures exhibit the symptoms of ordinary leiomyoma. different leiomyomas but be... Etiology and pathogenesis well-delineated, exhibit ample vascularity, contain numerous anechoic voids, which in case! Create diagnostic difficulties have not been well established the veins palpatory examination, these exophytic components were the common. Gave birth via cesarean section after an uneventful pregnancy conspicuous feature of justify. Of differentiation, the proper diagnosis is only rarely reached preoperatively, even when there is microscopic or macroscopic tumor.