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Adult Ocular and Orbital (Ocular Adnexa) Tumors
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
P.N. Plowman, Rachel Lewis, J.L. Hungerford
Smooth-muscle tumors are represented by leiomyomas, which are slow-growing, pale, and benign tumors. Leiomyomas are rare in the eye and have been described most frequently in the iris, with a few cases reported in the ciliary body. Re-evaluation of archival histological material suggests, however, that most so-called iris leiomyomas are really amelanotic benign melanocytic lesions. The only true ocular leiomyomas arise in the ciliary body, where they may grow quite large and are difficult to clinically distinguish from amelanotic melanomas. Ciliary-body leiomyomas are very circumscribed tumors that are easily excised from within the eye, the main indication for this procedure being exclusion of the alternative diagnosis of malignant melanoma.
Types of Fibroids
Published in John C. Petrozza, Uterine Fibroids, 2020
Even fibroids that appear grossly as “typical fibroids” can demonstrate heterogeneity in terms of mitotic activity, collagenous component, cellularity and fibrotic stroma [32]. Pathologically, the mitotic count has also been implicated in identifying fibroids on the spectrum of completely benign tumors to those of uncertain malignant potential and frank sarcoma [32]. Mitotic activity (greater than 10 or more mitoses per 10 high-power fields) has been one of the hallmarks of the diagnosis of leiomyosarcoma. In addition, cytologic atypia and tumor cell necrosis also play a role in diagnostic evaluation [18], and a further discussion of sarcomas will occur in Chapter 23. Smooth muscle tumors that have some features that are concerning for malignancy, but did not meet all diagnostic criteria, fall into the category of smooth muscle tumors of uncertain malignant potential [41].
Tumors of the Uterine Cervix and Endometrium
Published in Victor A. Bernstam, Pocket Guide to GENE LEVEL DIAGNOSTICS in Clinical Practice, 2019
The majority of leiomyomas display a DNA diploid pattern, whereas leiomyosarcomas exhibit a DNA tetraploid/polyploid or aneuploid DNA pattern. Although FCM evaluation of smooth muscle tumors is not recommended as a diagnostic tool, or for differentiation between benign and malignant tumors, its use for prognosis of the clinical behavior of these tumors may be of practical value. A combination of several histopathological characteristics usually improves the diagnostic accuracy and/or predictive value of FCM evaluations.
Long-term risk of uterine malignancies in women with uterine fibroids confirmed by myomectomy: a population-based study
Published in Journal of Obstetrics and Gynaecology, 2022
Another study by Hodge and Morton concluded that uterine sarcomas usually do not result from uterine fibroids, apart from rare cases. Now, there is a consensus among genetic studies that most sarcomas arise independently (Hodge and Morton 2007). Sarcomas usually have complex karyotypes and aneuploidy, whereas uterine fibroids have characteristic rearrangements, many of which are in common with other benign neoplasms (Stewart and Morton 2006). The intermediate types between these two patterns have not been described. Genetic studies of the origin of smooth muscle tumour of low malignant potential (STUMP) are rare. The study by Conconi et al., demonstrated the presence of a more similar genomic profile between STUMPs and leiomyosarcomas (Conconi et al. 2021). Histologic studies show rare cases of lesion progression from uterine fibroids to sarcoma (Robboy et al. 2000). The most convincing evidence comes from a rare subgroup of uterine fibroids with cellular or atypical histology based on data from two molecular genetics and two clinical studies (Christacos et al. 2006; Giuntoli et al. 2007; Taran et al. 2010; Hodge et al. 2014). In a clinical study, 3 of 18 patients with cellular or atypical variants perished from their disease (Giuntoli et al. 2007). There was a longer latent period between diagnosis and death in those patients who died from cellular or atypical variants of benign fibroids than in those with leiomyosarcoma (>6 years versus a median of 2.1 years). There are no data about how often cellular or atypical histologies show such abnormal clinical behaviour.
Recurrent Giant Cell Fibroblastoma in an Infant: A Diagnostic Challenge
Published in Fetal and Pediatric Pathology, 2022
Priyanka Maity, Uttara Chatterjee, Mou Das, Sabita Patra
The detailed paper on GCF by Enzinger et al, reported a misdiagnosis of sarcoma in 40% of cases [2]. In this case, the tumor at first excision was misdiagnosed as an embryonal rhabdomyosarcoma, which is the commonest soft tissue tumor of the paratesticular region in children and adolescents. Spindle cell RMS, considered to be a variant ERMS mainly occurs in the paratesticular structures [4]. This tumor is fairly cellular and is composed of interwoven bundles of elongated and atypical spindle cells with blunt ended, ovoid nuclei which somewhat resembles smooth muscle tumors. Spindle cell/sclerosing RMS is now thought to be a distinct entity, separate from ERMS and exhibits recurrent gene fusions involving NCOA2 or mutations of MYOD1(L122R) along with PIK3CA gene [5].
A rare case of orbital angioleiomyoma
Published in Orbit, 2021
Shiao Wei Wong, James Laybourne, Luciane Irion, Anne Cook
Angioleiomyoma is a rare variant of leiomyoma.10 It is a benign smooth muscle tumour derived from the muscular layer of blood vessels without elastic fibres.1 It is characterised by endothelium-lined vascular channels and a background of smooth muscle fascicles surrounding vascular lumina.10 Angioleiomyoma can occur anywhere in the body and can be found in the dermis, subcutaneous fat and fascia.1,11 It occurs most commonly on an extremity, particularly the lower leg.1,11 Hachisuga et al.11 reported an incidence of only 8.5% of angioleiomyomas in the head and neck. Based on Morimoto1, angioleiomyoma can be classified into three histological types, namely, solid (which is the most common), venous and cavernous. Morimoto1 also grouped these tumours into two anatomical groups. First, the larger group of extremity tumours, where a tumour is mainly of the solid type and is often painful due to stretching of nerves in the tumour (or its capsule) or release of mediators from mast cells. Pain may also be exacerbated by exposure to wind or cold.12 This group of mainly lower limb angioleiomyomas are twice as common in female patients. Second, the smaller group of head tumours, where the tumours are usually of the venous type and painless. This group of angioleiomyoma are more common in male patients.