Cutaneous leiomyomas and leiomyosarcomas: an immunohistochemical study with p53.
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Abstract:
Background: Cutaneous smooth muscle tumors are rare and sometimes the differential diagnosis between leiomyoma and leiomyosarcoma is difficult and based in very subtle criteria. We therefore tried to investigate the use of p53 in such a conundrum. This marker has rarely been reported in cutaneous leiomyomas and even in more rare occasions, in cutaneous leiomyomas. Material and Methods: We studied 30 benign cutaneous smooth muscle tumors, including angioleiomyomas, common leiomyomas and a symplastic leiomyoma, as well as four leiomyosarcomas and one cutaneous metastasis of leiomyosarcoma. All cases were reviewed in order to confirm the diagnosis, before the cases were included in the study. In all cases, we performed an immunohistochemical study in all cases with p53 and the percentage of positive cells was estimate counting a total of 1000 cells per case. Results: Six cases from the 31 (19.35%) benign cutaneous smooth muscle tumors showed some expression of p53. The expression of it varied from only occasional cells to 1% of the cells. On the contrary, all leiomyosarcomas investigated showed expression of p53, and in three of the four cases (75%), the marker was expressed by at least 80% of the tumoral cells. Only in one leiomyosarcoma, the marker was expressed by a low percentage (0.5%) of cells. No expression of p53 was found in the only case of symplastic leiomyoma, which was investigated. The case of a cutaneous metastasis of leiomyosarcoma showed expression of p53 by 20% of cells. Conclusions: We conclude that expression of p53 by a high percentage of cells in a cutaneous smooth muscle cell tumor should be considered as highly suspicious for malignancy.Keywords:
Smooth Muscle Tumor
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Inflammatory leiomyosarcoma, a rare entity first described in 1995, has been characterized by smooth muscle differentiation, a near-haploid karyotype, and a surprisingly good prognosis. The morphology is similar to that of conventional leiomyosarcoma admixed with a chronic inflammatory infiltrate. Thus far, only 15 cases have been reported in the English language literature. We report the clinical and pathological features of 3 additional cases of inflammatory leiomyosarcoma. Two women (ages 64 and 25, respectively) and 1 man (age 32) presented with a thigh, ovary, and lung mass, respectively. Inflammatory symptoms, such as anorexia, fever, night sweats, abdominal pain, and diarrhea, coincided with the thigh and ovarian primaries. Immunohistochemical studies revealed diffuse positivity for desmin and poor expression for other smooth muscle and skeletal muscle markers (muscle-specific actin [0/3], alpha-smooth muscle actin 1/3 [focal], calponin [1/3], caldesmon [0/3], and myogenin [0/3]). CD68 was diffusely positive in both the histiocytes and spindle cell component in all cases. Ultrastructural evaluation of 1 case (lung primary) lacked definitive smooth muscle differentiation. Cytogenetic analysis in 1 of 2 cases that were karyotyped, identified a near-haploid karyotype, which has been reported in other cases of inflammatory leiomyosarcoma. The other case showed 2 clonal populations of cells with interstitial deletions of the short arm of chromosome 8 and the long arm of chromosome 9, respectively. The case without cytogenetic data was intimately associated with an ovarian mature teratoma. These data also suggest that inflammatory leiomyosarcoma may lack smooth muscle differentiation, characterized by diffuse immunoreactivity for desmin but lack of immunoreactivity for alpha-smooth muscle actin, calponin, and caldesmon. In addition, 2 of the 3 cases developed distant metastases to the lungs, which suggests that these lesions may have a worse prognosis than previously believed.
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The most common mesenchymal tumours of the uterine corpus originate from smooth muscle cells. Leiomyomas are commonly found in women of child bearing age; however, leiomyosarcomas occur later in life (50-55 years of age). Most uterine leiomyosarcomas occur de novo, but rare cases of leiomyosarcomas that arise from leiomyomas have been reported. We present two cases of fertile women with submucosal leiomyomas that became malignant and discuss their pathologic features and immunohistochemistry studies for P16, P53 and Ki67.
Uterine Leiomyoma
Smooth Muscle Tumor
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Summary Mast cells (MCs) have been reported in the myometrium and uterine smooth muscle tumors. We examined the number of MCs in various uterine smooth muscle tumors (including leiomyosarcomas) and assessed whether this feature might be of value in their pathologic diagnosis. The number of MCs in 95 uterine smooth muscle tumors, including 55 ordinary leiomyomas, 17 cellular leiomyomas, 8 bizarre leiomyomas, and 15 leiomyosarcomas, was counted using toluidine blue and immunohistochemical staining. The number of MCs that stained for tryptase was lowest in leiomyosarcoma and next lowest in ordinary leiomyoma; the number in each of these two groups was significantly lower than in the myometrium (p < 0.001). In cellular and bizarre leiomyomas, the number of MCs was significantly higher than in ordinary leiomyoma (p < 0.001 and p < 0.001, respectively) and leiomyosarcoma (p < 0.001 and p < 0.005, respectively). Statistical analysis revealed that counting the number of MCs and using a cut-off value of 16 MCs per high-power-field is useful for the differential diagnosis of leiomyosarcomas from cellular leiomyoma and bizarre leiomyoma, yielding 100% sensitivity and 96% specificity. The number of MCs was significantly lower in leiomyosarcomas at TNM stages III and IV than in those at stages I and II (p < 0.05), but there was no significant correlation between the number of MCs and patient survival. These results suggest that the number of MCs might be useful as part of a multivariate approach to the differential diagnosis of leiomyosarcoma from bizarre or cellular leiomyoma.
Myometrium
Smooth Muscle Tumor
Uterine Leiomyoma
Tryptase
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Cutaneous meningiomas are divided into three groups. Type I lesions present at birth and are derived from ectopic arachnoid cells. Type II lesions usually present in adults and are derived from arachnoid cells surrounding nerve bundles. Type III lesions are due to direct extension or metastasis from dural-based neoplasms. Dural-based meningiomas are known to express p63. The aim of our study is to examine the expression of p63 in type II and type III meningioma. Two cases of cutaneous meningioma (type II and type III) were evaluated for the expression of p63, EMA, CK 5/6, S100 and CD31. The cells of interest were spindled to epithelioid and arranged in a whorling pattern. Immunohistochemical staining showed expression of EMA and p63 in both cases, while stains for CK 5/6, S100 and CD31 were negative. Among cutaneous tumors, p63 is considered a marker of epithelial derivation, as it is positive in epidermal and adnexal neoplasms. It is important to be aware of p63 expression in the context of cutaneous meningioma to avoid misinterpretation as an epithelial tumor. On the basis of our small study, it is unlikely that p63 expression would be helpful in distinguishing between type II and type III meningioma, as both may be p63-positive.
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Objective: To investigate the clinic pathological features diagnosis main point and prognosis of cutaneous leiomyosarcoma(CLMS).Methods: Histopathology,immunohistochemical stainings observation were analyzed in two cases of CLMS and the related literatures were reviewed.Results: Case 1 was subcutaneous leiomyosarcoma with tubercular growth pattern,rich tumor cell,big heterogeneous type,active mitotic;Case 2 was dermis leiomyosarcoma with diffuse growth pattern,few tumor cell,well differentiated,no more mitotic.Immunohistochemically,the two cases reacted positively with muscle action、MSA and Vim,Case 1 also expressed desman partially.The two cases were revisited to date,no recurrences and metastases.Conclusion: Cutaneous leiomyosarcoma is a rare tumor,subdivided into dermis and subcutaneous forms because of their different tissue origins and prognosis features.We must discriminate between them.Diagnosis need synthetic appraisal besides mitotic counts and smooth muscle tumor of uncertain malignant potential should be used for diagnosis of certain cases.Primary treatment for cutaneous leiomyosarcoma is surgical excision.
Histopathology
Smooth Muscle Tumor
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Objective: To investigate the clinicoal pathology of uterine smooth muscle tumors. Method: Clinical and pathological data of 200 cases of uterine smooth muscle tumors were analyzed retrospectively. Result : The youngest who suffers from uterine leiomyoma is 24 years old ,the oldest is 61, the mean age is 43.3 .And the ones whose age is between 41 and 50 have the highest happening rate. Uterine smooth muscle may coexist with other diseases such as endometrial hyperplasia ,ademomyosis ,all kinds of cysts of ovary ,all kinds of benign or malignant tumours et al. Among 200 cases of the tumors ,there were 196 cases of usual leiomyoma ,3 smooth muscle tumor of uncertain malignant potential and 1 leiomyosarcoma. Conclusion : The uterine leiomyoma is easy to be found and may coexist with all kinds of disease of uterus and ovary . Nuclear mitotic activity is an important but not sole criterion in the diagnosis of benign or malignant uterine smooth muscle tumors;the combination of cellular atypia,margin infiltration,clinical data should also be taken into consideration.
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Atypia
Uterine Leiomyoma
Nuclear atypia
Endometrial hyperplasia
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The clinical and pathological findings of six cases of leiomyosarcoma arising from blood vessels of different caliber are described. The term vascular leiomyosarcoma, having both a topographic and morphologic significance, is proposed for these tumors. The histologic pattern is characterized by a proliferation of atypical smooth muscle cells with a large number of intermingled blood vessels. Mitoses were counted per 10 high power field (hpf) and tumors were divided in three groups: group I, 10 to 20 mitoses, group II, 20 to 35 mitoses, and group III, more than 35 mitoses per 10 hpf. The mitotic index seems to be the most important pathological feature on which a prognostic evaluation for vascular leiomyosarcomas can be based. Tumors in group I had neither local recurrences nor metastases; the one tumor in group II had one local recurrence, but the patient is free of disease 6 years after surgical treatment; the three tumors in group III developed distant metastases and constitutional symptoms. Vascular leiomyoma, bizarre leiomyoma, and hemangiopericytoma are included in the differential diagnosis of vascular leiomyosarcoma. The possibility that vascular leiomyosarcoma arising from small vessels represents the malignant counterpart of vascular leiomyoma is proposed.
Smooth Muscle Tumor
Hemangiopericytoma
Mitotic index
High-power field
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Leiomyosarcoma in the pediatric age group is uncommon and incompletely characterized. A series of 20 primary leiomyosarcomas of soft tissue occurring in children younger than 16 years is presented. No significant gender predilection was observed (11 girls and 9 boys). Patient age ranged from 4 to 15 years (median, 12 years). Tumor size ranged from 0.5 to 13 cm (median, 2.5 cm); subcutaneous and deep locations were equally represented. Tumors were evenly distributed among the trunk (30%), head and neck (25%), lower limbs (25%), and upper limbs (20%). All lesions showed at least focally typical features of smooth muscle differentiation, principally in the form of fascicles of eosinophilic spindle cells with cigar-shaped nuclei. An unusual whorled growth pattern was seen in two cases. Morphologic variants including inflammatory leiomyosarcoma (one case), granular cell leiomyosarcoma (two cases), giant-cell rich leiomyosarcoma (two cases), and epithelioid leiomyosarcoma (one case) were seen. Dystrophic calcifications were present in two cases. Most lesions (85%) were low grade. Immunohistochemical staining showed positivity for alpha-smooth muscle actin in 89% of the cases, HHF-35 in 87%, and desmin in 61%. Positivity for cytokeratins, observed in 6 (43%) of 14 cases tested, was usually strong and was diffuse in two cases. Follow-up data, available in 15 (75%) patients (median duration, 49 months), showed late local recurrence in only two cases, one with progression to a higher grade lesion, and no metastasis. These results show that, although extremely rare, soft-tissue leiomyosarcomas do occur in children, in whom they usually present as small morphologically low-grade lesions that seem to behave in a relatively indolent fashion, although longer follow-up data are needed. Differential diagnosis in this setting includes infantile myofibromatosis, leiomyoma, monophasic synovial sarcoma, and spindle cell rhabdomyosarcoma.
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Uterine Leiomyoma
Smooth Muscle Tumor
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One hundred smooth muscle tumors arising in the gastrointestinal tract and retroperitoneum were reviewed in an attempt to define criteria for the diagnosis of leiomyosarcoma in these sites. On the basis of aggressive behavior, 56 of these neoplasms were diagnosed as leiomyosarcoma. Mitoses were found to be the most useful indicator of malignancy; all of the tumors with five or more mitoses/10 HPF behaved aggressively and smooth muscle tumors with this degree of mitotic activity should be diagnosed as leiomyosarcoma. A paucity of mitoses, however, is no assurance of benignity as nearly 40% of the leiomyosarcomas in this series had fewer than five mitoses/10 HPF. Tumor cell necrosis was closely associated with aggressive behavior even when mitoses were infrequent and it is doubtful that benign smooth muscle tumors develop extensive tumor cell necrosis. In the absence of the requisite number of mitoses or tumor necrosis, it is difficult to distinguish some leiomyosarcomas from leiomyomas, but tumor size, cellularity and cellular atypia may be helpful parameters when assessed together. The importance of these criteria in different anatomical sites is discussed. It is emphasized that the criteria for the diagnosis of leiomyosarcoma of the uterus do not apply to non-uterine smooth muscle tumors. The actuarial 2-year survival rate was as follows: gastric leiomyosarcoma, 40%; small intestinal leiomyosarcoma, 60%; and retroperitoneal leiomyosarcoma, 16%.
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Atypia
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