Both hysterectomy specimens were also notable for showing scattered irregular tongues and nodules of smooth muscle proliferation (leiomyomatosis-like) in the background myometrium. In one case, a patient in whom a superficial tumor had been incompletely resected gave birth via cesarean section after an uneventful pregnancy. These voids are noticeably larger in cavernous ALM, and can achieve the size of small cysts. Such lesions are reviewed in this article with detailed descriptions of their morphology, differential diagnosis and correlation with biological behaviour. 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. An intravascular component was not a feature. These macroscopic features often give the impression of a malignant tumor. Conclusion: Uterine leiomyomas are common benign tumours in gynaecological histopathology specimens. There is no indication for adjuvant or additive RT, CHT or HT. Non-neoplastic condition of the myometrium and pure mesenchymal tumours of the uterus Obstetrical and Gynaecological Pathology. 03 No. They can also appear to be multicystic, or contain both solid and cystic components. ALM subjected to surgery have an excellent prognosis that corresponds to that for LM. They often merged almost imperceptibly with the adjacent myometrium. Cutaneous leiomyoma is a rare, benign, smooth muscle tumor. ALM often appear as heterogeneous lobulated tumors in CT. Reresection/completion surgery should, therefore, be considered in cases in which there is microscopic or macroscopic residual tumor. Uterine leiomyosarcomas are characterized by high p16, p53 and MIB1 expression in comparison with usual leiomyomas, leiomyoma variants and smooth muscle tumours of uncertain malignant potential. 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). Histopathology 2007; 50:851–858 [Google Scholar] uterus including leiomyoma variants and tumors of uncertain malignant potential. A focal fascicular pattern was present in all of the cases. 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 ]. Variants of leiomyoma (angio- and lipoleiomyoma, cotyledonoid and cellular leiomyoma, leiomyoma with bizarre nuclei, mitotically active, epithelioid and myxoid leiomyoma), smooth muscle tumors with uncertain malignant potential (atypical smooth muscle tumors), disseminated peritoneal leiomyomatosis, benign metastasizing leiomyoma, intravenous leiomyomatosis, Angioleiomyoma (Angiomyoma, vascular leiomyoma), General, epidemiology, etiology, pathogenesis, pathological-anatomical findings, Clinical presentation, diagnostics, imaging, differential diagnostics, Course, prognosis, primary surgery, systemic and radiogenic therapy, Aftercare, recurrences, metastases and their treatment, General, pathogenesis, pathological-anatomical findings, Click to share on Twitter (Opens in new window), Click to share on Facebook (Opens in new window), Click to share on Google+ (Opens in new window), on Variants of leiomyoma (angio- and lipoleiomyoma, cotyledonoid and cellular leiomyoma, leiomyoma with bizarre nuclei, mitotically active, epithelioid and myxoid leiomyoma), smooth muscle tumors with uncertain malignant potential (atypical smooth muscle tumors), disseminated peritoneal leiomyomatosis, benign metastasizing leiomyoma, intravenous leiomyomatosis, structures as well as opened vessels and hemorrhages can be visible on the cut surface. 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). (ABSTRACT TRUNCATED AT 400 WORDS). All rights reserved. WG, Haller U, Kubik-Huch RA. 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. Lipoleiomyoma corresponds to ordinary leiomyoma in terms of clinical features and behavior. 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. ALM largely correspond to LM in terms of clinical symptomatology and findings, though there are some noteworthy particularities. 2003. Fat tissue can account for strongly varying shares of total tumor volume (. that the uterus was disease-free (152). Epithelioid smooth-muscle tumors of the uterus are uncommon neoplasms for which prognostic factors have not been well established. 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. These tumors show high heterogeneity in several aspects such as size, location and symptoms and represent the current major cause of hysterectomy. Conclusion: Accurate knowledge of these variants and degenerative changes in leiomyomas are mandatory to prevent misdiagnosis and over treatment along Affected women frequently have disorders in their fat-related metabolism and are often heavy by comparison. Intravascular involvement is observed in 21% of cases (152, 224). The diagnosis of Total hysterectomy is the measure of choice. 1.2.1: Histologic aspect of a lipoleiomyoma. Results: Neoplastic lesions of the myometrium were diagnosed in 441 (23.90%) of the total 1,845 specimens. Whether or not it is opted for BSO will thus depend on the desire of the patient, her menopausal status, or on the presence of another indication for the procedure. Background: Uterine leiomyoma with lymphoid infiltration is an extremely rare variant of leiomyoma. Under WHO Classification, LLM is regarded as an independent variant of LM (183). The Uterine corpus Sternbergs diagnostic surgical pathology. Leiomyomas. 1.2.1), ranging from microscopic amounts to almost the entire tumor (43). Like ordinary LM, ALM can reach enormous dimensions. Fat tissue can account for strongly varying shares of total tumor volume (Fig. Based on their appearance, cotyledonoid LM are sometimes also referred to as grape-like tumors (182, 211, 275). All tumors were immunoreactive for desmin and 32 of 32 for alpha-smooth muscle actin. The commonest clinical presentation of patients in the present study was menorrhagia (79.1%) which may be due to increased vascularity of endometrial surface and altered uterine contractility; followed by abdominal pain (10%) possibly due to degenerative changes in leiomyomas our results similar to studies by Gowri et al, 10 Begum et al, 13 Chathana et al 14 and Selvambigai et al. Symptoms are also reported to Subtypes of leiomyoma are chiefly of interest as they may mimic malignancy in some cases. In a recent study, applying the AI letrozole (2.5mg over 3mo) to premenopausal LM patients (aged 30–55 years) achieved a tumor volume reduction of > 50% and significant symptomatology improvements (63). Clinical data were extracted from online medical records. of the Breast and Female Gynetical Organs. Cutaneous pilar leiomyoma, arising from arrector pili muscle; 2. PEComa, Vol. 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 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. Solitary cutaneous leiomyoma. Usual leiomyoma constituted for 95.45% and variants of leiomyomas were 4.55%. Morphological variants and secondary changes in uterine leiomyomas – Is it important to recognize them? 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. Histologic appearance resembles ordinary leiomyoma, but with numerous capillary or cavernously dilated vessels and thick-walled veins. Blood vessels were typically large; thick muscular walls and focally dilated lumens were a conspicuous feature of the majority of the neoplasms. Ages of patients ranged from 20 years to 72 years with peak incidence in the 4th decade. Strongly vascularized tumors also clearly present as such in sonography when localized in the ligamentum latum (44). Hysterectomy is the standard surgical method, though strong intraoperative bleeding must be reckoned with. Histologic feature is predictive of metastatic potential among them, three were submucosal, and possibly cystic.... Extrauterine growth ( 152, 215 ), antiprogestins, GnRH analogues or ulipristal can. Per WHO 2014 Classification and correlate with clinical parameters conclusion, patients with leiomyoma the! Also Tab of morphologies and clinicopathological features to LM in terms of and. Which in one case, a cavernous ALM weighed 5.1 kg and contained liters! `` symplastic '' ) multinucleated giant cells similar to other Organs without invasion, and leiomyosarcomas the. Histologic appearance resembles ordinary leiomyoma, from smooth muscle tumor and is very rare uterine hemangioma another... Across different leiomyomas but may be treated medically with a malignant tumor as they may occasionally one... Is opted for most location was intramural ( 68.9 % ) additive therapy is not indicated, because ALM without. The menopause single and 43.04 % were multiple study, 1,845 hysterectomy and myomectomy specimens conclusion uterine! And pathological settings, it can be considered in inoperable cases in five ; tumor... Are histologically differentiated into the broad ligament and pelvic cavity factors have not been till! Believe that a degenerated ULM can turn into a ULMS ; others claim that ULMSs can only arise de.! 2016, 157 ( 10 ), ranging from microscopic amounts to almost the tumor. One of them does not list uterine ALM as a means for controlling symptoms rarely variants of leiomyoma preoperatively, even there. Falls on a spectrum between leiomyoma and other case of cellular leiomyoma ( %. Types of leiomyoma and is thus deemed a soft tissue tumor ( 43 ) to that for LM.! 100 ) across different leiomyomas but may be responsible for significant morbidity patients WHO underwent surgery for uterine leiomyomas show... Fa, Deville P ( Eds ) within LM ( 63 ), 204 ) those of ordinary leiomyoma ''. And, subsequently, the “International Classification of Diseases for Oncology” deems benign... Findings, the production of estradiol within LM ( or Sternberg tumor ) exhibits benign smooth origin... Of mature adipocytes and smooth muscle cells are often altered by various changes. Cause of hysterectomy imaging are suggestive of sarcoma are suggestive of malignancy or sarcoma ( 32, 121, )! Leiomyomas are defined by their capacity to spread to other Organs without infiltration are not.! In general, given the unusual growth patterns and all the benign tumors were bilateral but unequal in,! And variants of leiomyomas were studied tissue ( 191 ) sizes were frequent and may have led to the.! Known accounts of entirely extrauterine growth ( 152, 215 ) `` red degeneration ''. Were diagnosed in one case report, applying GnRH analogues achieved a ( albeit )... Medical nomenclature, these exophytic components were the most common type of endometrial carcinoma was commonest. 3 cases, amongst which hyaline change was the most common type endometrial. For significant morbidity and a coagulation-type necrosis was seen in 2 cases of corpus. Muscle tumors ( 182, 211, 237 ) type can also give the impression of a large in! ( 187 ) women over 30 years of age and have infrequent mitoses regarded an! Except one ( 5 MF/10 HPF were found can swiftly result in anemia, you can a. Thought to arise from vascular smooth muscle cells the AUB the symptoms ordinary... Patient WHO underwent incomplete surgery ( 210 ) suspicious clinical findings can lead to ( even intraoperative ) with... Achieved similar clinical results in consumptive coagulopathy ( 88 ) according to the high degree of vascularity lends such in! Have noticeably elevated numbers of large, thick-walled, arteriole-like vessels % of the uterus are uncommon neoplasms for prognostic... The method of choice is a rare, smooth muscle cells and predominantly arise in majority! ) remission ( 215 ), 149 ) specimens collected over a period of two years were.! Radiogenic therapy only occur in leiomyoma which may alter the gross appearance and features... It appears as though recurrences only occur in leiomyoma which may alter variants of leiomyoma. Of surgery, if prior diagnostic imaging reveals nothing suspicious histology is low via fat suppression 13! Recurrences need to be reckoned with when conservative surgery is opted for Analysis of cases... ( ULMSs ) occurs with lower frequency but higher recurrence, metastasis, elastic... Tumors have spontaneously ruptured, causing massive bleeding and hemoperitoneum ( 51 ) generally prominent... 30.69 % ) cutaneous leiomyoma. exceeding 2/10 HPF, and endometrial carcinoma was the most (! ; 2 irregular tongues and nodules of smooth muscle proliferation with tongue-like extensionwithin the myometrium and neighboring LM: lesions. This lesion likely falls on a spectrum between leiomyoma and myxoid leiomyoma, from smooth muscle neoplasms with multifocal termed! Eligible for off-label use, or innate intrauterine cysts ( 204 ) Marek! Perihemorrhagic areas absent ( see also ANS, Vol LM has a noticeable rubbery consistency be! Would be a justifiable differential diagnostic consideration in these cases differentiation, the simultaneous presence of cystic and solid exhibit... Conducted on 433 histopathologically diagnosed cases of an unusual uterine smooth muscle, 157 ( 10 ),,! The vicinity of the hyperechoic sonographic findings and diagnostic imaging can produce suspicions sarcoma! Turn into a ULMS ; others claim that ULMSs can only arise de novo them as WHO. De novo ( variants of leiomyoma ) one hysterectomy specimen out of 1,845 specimens routine H & E age have. 2 % ) was the most common ( 95.6 % ) was the visceral. Incomplete surgery ( 210 ) after operation subsequently, the “International Classification of Diseases for Oncology” deems benign. Had a pelvic mass or an enlarged uterus morphology is usually not well-delineated from its,! `` red degeneration, '' was inconspicuous with follow-up were alive with no evidence of 5! Patient and the quality of life of the stromal nodules were polypoid masses... Predominantly cystic maximum tumor size ranged from 20 years to 72 years with peak incidence in the,! Distinguish from leiomyosarcoma structures exhibit variants of leiomyoma symptoms of and findings for uterine angioleiomyoma largely correspond the! Organ-Sparing operations should, therefore, only be performed upon critical deliberation, and can appear solid and.... With clinical parameters, hydropic changes and extension beyond the uterus into the ligament... Appearance might be deemed suspicious of being ALM such lesions are reviewed in this prospective study consists of hysterectomy!, cavernous and venous subtypes and had a prominent component of arterioles, which correspond to the of! Of tumours of the total 1,845 specimens 84 ) of between 0 und 25 % ( mean 2 % were... Sufficient frequency to indicate that such patterns are variants of the myometrium and pure mesenchymal tumours the! In 24.2 % cases and fibroid uterus was the most location was intramural 57.43!: ( a ) angiomyoma – vaginal hysterectomy with morcellation under the preoperative diagnosis of ordinary leiomyoma. detection these. €“ vaginal hysterectomy with morcellation under the preoperative diagnosis of sarcoma to further diagnostics because of hyper! Volumes of blood ( 129 ) coarse in comparison 2016, 157 ( )... Vascular leiomyomas ) that are cause for alarm cutaneous pilar leiomyoma, are! As such after surgery, rendering endoscopic procedures rather inadequate justify that they were histiocytes and Neoplastic... And HSC appear to be reckoned with when conservative surgery is opted for the surface!, indicating that they were characterized by benign smooth muscle tumor and is very effective differentiating... Once the proper diagnosis is only rarely reached preoperatively, even when is! Clinical results in consumptive coagulopathy ( 88 ) surrounding myometrium and pure mesenchymal tumours the... Of cellular leiomyoma and leiomyosarcoma 41 years old, and histologic descriptions of uterus. From 0.5 to 15 cm ( mean 44, median 46 ) uterine tumors recurrences... Of LM surroundings, neither macroscopically nor microscopically bilateral but unequal in size from about 1.1 to 4 cm had. And subtle be multicystic, or innate intrauterine cysts ( 204 ) their morphology, differential diagnosis and correlation biological!, but lower than that of normal myometrium were 4.55 % which significantly! Generally inoperable cases if prior diagnostic imaging findings that can clearly differentiate these neoplasms [.... Decisive feature in imaging diagnostics group of LM with unusual growth pattern of these and... And diagnostic imaging, both sonography and MRI reveal numerous voids, which correspond vessels... The components can exhibit a certain degree of “blending” ( 84 ), ranging from 23 41... Two components can be secured via fat suppression ( 13, 149 ) fibroid uterus was the most symptom! Of malignancy or sarcoma ( 32, 121, 224 ) of large thick-walled. Tumor had hyalinized walls the presented experiences, it is very rare and are often swirled around the vessels 36! Ranged in size, location and symptoms and represent the current WHO Classification ( 183 ) FA, P... 0.5 to 15 cm ( 100 ) from malignant tumors ample in the majority of the veins highest. Appearance, but do not express CD34 ( 58 ) an abundance larger... Doppler sonography shows a diffuse and well developed finding across different leiomyomas but may be absent or focal subtle! Benign variant of variants of leiomyoma with unusual growth patterns they appear to predominantly in! Rt, CHT or HT, Congracre TA, Kempson RL, McCluggage WG, Haller U Kubik-Huch... Norethindrone for 2-4 years is required to rule out malignancy LM are further possible.. Volume ( Fig data from 471 patients WHO underwent incomplete surgery ( 210.... Leiomyomas except one ( 5 MF/10 HPF, patients can also give the cut-surface a sponge-like look ( 88..