Solitary Pilar Leiomyoma of the Nasal Dorsum: Case Report and Literature Review

Cutaneous leiomyoma is an infrequently occurring benign tumor that arises from smooth muscle in the skin. Pilar leiomyoma, a subtype of cutaneous leiomyoma, arises from the arrector pili muscle associated with hair follicles. Pilar leiomyoma, particularly in the head and neck region, is rare and frequently misdiagnosed. We report one patient who developed pilar leiomyoma of the nasal dorsum, explore the differential diagnosis and review the characteristics of previously reported individuals with pilar leiomyoma of the nasal dorsum.     

Positive correlation of vanilloid receptor subtype1 and prostaglandin E2 expression with pain in leiomyomas

Cutaneous leiomyomas are benign smooth muscle tumors that are occasionally painful. The mechanism of pain related to leiomyoma is not fully understood. To investigate the possible involvement of algoneic factors in pain from cutaneous leiomyomas. We present a case of cutaneous leiomyoma with severe, diffused pain in a large area and collected 10 more specimens of cutaneous leiomyoma with or without pain in patient histories. We immunohistochemiacally examined the expression of algoneic factors: serotonin, histamin, Substance P, PGE2, BDKRB2, VR1 and CGRP. We compared the pain area and expression of algoneic factors to reveal possible correlations. We describe here a patient with a cutaneous leiomyoma 1‐cm in diameter, which caused severe pain diffused throughout an area of 20‐cm around the tumor. The pain completely resolved after surgical excision of the leiomyoma. We observed that the leiomyoma cells expressed CGRP, PGE2 and VR1 in this case. We found a positive correlation between VR1 and PGE2 expression in the leiomyoma cells and areas with pain around the tumors among 11 specimens in total. VR1 and PGE2 might be key algogenic substances in painful leiomyoma.     

Nipple leiomyoma: A rare neoplasm with a broad spectrum of histologic appearances

Cutaneous leiomyomas are rare benign smooth‐muscle tumors. These lesions are distinguished based on their cell of origin and are subclassified as pilar leiomyoma, angioleiomyoma, and genital‐type leiomyoma. Nipple leiomyoma is the least common genital‐type leiomyoma, arising from the dartoic muscle cell of the nipple. Histologic examination of the lesion is necessary for definitive diagnosis, and these uncommon tumors can pose a diagnostic challenge. We describe herein a series of six nipple leiomyomas with a spectrum of histologic appearances.     

Association of multiple familial cutaneous leiomyoma with a uterine symplastic leiomyoma

We describe a patient who has familial cutaneous leiomyoma in association with a symplastic uterine leiomyoma. This association has not been described previously.     

Germline fumarate hydratase mutations in families with multiple cutaneous and uterine leiomyomata

Germline mutations in the fumarate hydratase gene (FH) predispose to multiple cutaneous and uterine leiomyoma syndrome (MCL) and MCL associated with renal cell cancer. MCL is inherited in an autosomal dominant pattern, manifesting as skin leiomyoma and uterine fibroids in affected individuals. Fumarate hydratase, a component of the tricarboxylic acid cycle, acts as a tumor suppressor gene in the development of cutaneous and uterine leiomyoma and renal cell cancer in this syndrome. Here we report the clinical and mutational analysis of five families with MCL, with the identification of five new mutations affecting highly conserved residues of the FH protein. These results provide further evidence for the role of the FH gene in the pathogenesis of MCL.     

Genital leiomyoma: surgical excision for both diagnosis and treatment of a unilateral leiomyoma of the male nipple

We describe a rare case of unilateral leiomyoma of the nipple in a man presenting with pruritus of the nipple for 3 months. A conservative surgical excision is performed for diagnosis. Histologic examination and immunostaining confirmed the diagnosis of leiomyoma. The leiomyoma is completely excised, and the patient denies pruritus after surgery. Conservative surgical excision of a persistently hard and pruritic nipple can be effectively used as both diagnostic measure and treatment modality.     

Genital leiomyomas of the male nipple: two cases

BACKGROUND: Genital leiomyoma is a rare benign solitary skin tumor, not painful, developed from smooth muscle. Genital leiomyoma arising from the nipple is extremely rare, especially in males.CASES REPORT: A 47 year-old male had a 2 cm cutaneous plaque with nodules located on the right nipple. This plaque was circumscribed, erythematous, pruriginous and was not painful. The lesion had been noticed by the patient two years ago. A 37 year-old male showed a 1.5 cm cutaneous plaque located on the left nipple. The plaque was slightly erythematous, pruriginous, not painful and had been noticed by the patient 5 years earlier. Histology provided the diagnosis of genital leiomyoma in both cases. No surgical therapy was performed.DISCUSSION: Cutaneous leiomyomas are classified in 3 types regarding their origin: multiple or solitary piloleiomyoma, arising from arectores pilorum muscles, solitary genital leiomyoma, arising from the dartoic, vulvar, or mammillary muscles, and solitary angioleiomyoma, arising from the vein muscles. Clinically, genital leiomyoma is a 1 cm diameter solitary erythematous, firm nodule. According to many authors, genital leiomyoma is asymptomatic, but in the 2 patients, the lesions were pruriginous. Surgical excision is usually performed.     

Hyperkeratotic lesion of the nipple revealing cutaneous leiomyoma

BACKGROUND: Leiomyoma of the nipple and areola is a rare benign neoplasm. We report the case of a patient with leiomyoma of the nipple presenting as a hyperkeratotic plaque. OBSERVATION: A 23-year-old patient presented with a five year history of a papillomatous, hyperkeratotic, painful plaque originating in her right nipple. Histological examination of a punch biopsy showed hyperkeratosis of the epidermis with dilatation of the lymphatic vessels within the dermis. Surgical excision revealed a proliferation of smooth muscle fibres, leading to diagnosis of leiomyoma. DISCUSSION: The clinical and histological features were initially consistent with idiopathic naevoid hyperkeratosis of areola. However, associated pain is uncommon in idiopathic lesions. This unusual feature led us to surgical excision enabling the diagnosis of leiomyoma. A hyperkeratotic lesion of the nipple may be associated with benign or malignant neoplasms, hamartoma or chronic dermatoses, or it may be idiopathic. In the present case, the hyperkeratotic lesion revealed subareolar leiomyoma. This is an uncommon clinical presentation not previously seen in medical observations, since leiomyoma usually presents as a firm, painful lump in the subareolar region.     

Incidence of mature adipocytic component within cutaneous smooth muscle neoplasms

Angioleiomyoma is a benign smooth muscle tumor of the subcutis. The presence of mature adipocytes has been described in this tumor under the rubric of ‘angiolipoleiomyoma’ or, erroneously, ‘angiomyolipoma’ (these are not PEComas). Previous studies have found adipocytes in only 2–3% of angioleiomyoma. Anecdotally, the incidence appeared to be greater than this in our practice. Moreover, the presence of adipocytes has not been evaluated in pilar leiomyoma or cutaneous leiomyosarcoma. We searched the pathology archives from 2007 to 2014 for all cutaneous and subcutaneous leiomyoma and leiomyosarcoma; cases were reviewed to confirm the diagnosis and evaluate for mature adipocytes. Seven of 73 total cases (10%) contained mature adipocytes: 1 of 33 pilar leiomyoma (3%), 4 of 22 angioleiomyoma (18%) and 2 of 18 leiomyosarcoma (11%). In our series, the 18% incidence of ‘angioleiomyoma with fat’ (our preferred terminology) is higher than the previously reported incidence of 2–3%. We also report the rare presence of mature adipocytes within pilar leiomyoma and cutaneous leiomyosarcoma, a finding not previously reported to our knowledge. Mature adipocytes may be present within cutaneous and subcutaneous leiomyomas and leiomyosarcomas and should not detract from the diagnosis or lead to concern for an adipocytic neoplasm or PEComa.     

Zosteriform cutaneous leiomyoma-Type Ⅱ: An uncommon presentation

Cutaneous leiomyomas are rare, benign smooth muscle tumors, characterized by painful nodules in most of the cases. They can occur in multiple disseminated, segmental or zosteriform and solitary forms. Segmental or zosteriform leiomyoma can occur either alone(Type Ⅰ), or with scattered nonsegmental lesions elsewhere(Type Ⅱ); the latter variety occurring rarely. Here we present a case of Type Ⅱ zosteriform leiomyoma in a middle aged individual.     

Two cases of male nipple leiomyoma: idiopathic leiomyoma and gynecomastia-associated leiomyoma

We describe 2 cases of male nipple leiomyoma. A 70-year-old man had a painful subcutaneous tumor on his left nipple of 6 months duration. Histopathology disclosed dermal spindle cells with oval-shaped nuclei forming interlacing bundles with irregular pattern. Glandular elements were absent. The spindle cells were positive to α-smooth muscle actin, desmin, and vimentin. Estrogen receptor (ER) and progesterone receptor (PrR) were negative. We diagnosed this case as male leiomyoma of the nipple. Another patient was a 61-year-old man with gynecomastia induced by spironolactone of 6 months duration. He also had a painful nodule on his left nipple and histopathology disclosed spindle-shaped tumor cells as in the previous patient. The tumor was accompanied by glandular elements in the deep dermis and subcutaneous tissue, which showed apocrine secretion and were positive for α-smooth muscle actin, ER, and PrR. These glandular elements were interpreted as mammary gland. But ER and PrR stain did not show positive results for leiomyoma in the upper dermis. To the best of our knowledge, this is the first report of male idiopathic and gynecomastia-induced leiomyoma with ER and PrR staining.     

育龄子宫肌瘤患者子宫内膜炎症因子和激素水平与纤维化的关联

目的探讨育龄子宫肌瘤患者子宫内膜炎症因子和激素水平与纤维化的关联。方法选取2016年1月至2019年1月期间十堰市妇幼保健院诊治的150例育龄期子宫肌瘤患者作为研究对象。采用实时荧光定量PCR法测定患者子宫肌瘤组织、周围组织中炎症因子IL-17、雌激素受体ESR1、ESR2,以及纤维化因子MMP9、TGF-β3 mRNA相对表达值。根据育龄子宫肌瘤患者子宫内膜组织IL-17 mRNA相对表达值,将其分为高表达组与低表达组,比较两组患者雌激素受体ESR1、ESR2以及纤维化因子MMP9、TGF-β3 mRNA相对情况。采用LOGSTIC多元回归分析子宫内膜炎症因子IL-17与ESR1、ESR2、MMP9、TGF-β3的相关性。结果子宫肌瘤组织中IL-17 mRNA相对值高于周围组织,差异具有统计学意义(P<0.05)。子宫肌瘤组织中ESR1、ESR2 mRNA相对值高于周围组织,差异具有统计学意义(P<0.05)。子宫肌瘤组织中纤维化因子MMP9、TGF-β3 mRNA相对值高于周围组织,差异具有统计学意义(P<0.05)。IL-17 mRNA高表达组患者ESR1、ESR2 mRNA相对值高于低表达组,差异具有统计学意义(P<0.05)。IL-17 mRNA高表达组患者纤维化因子MMP9、TGF-β3 mRNA相对值高于低表达组,差异具有统计学意义(P<0.05)。Logistic多因素回归分析显示,ESR1、ESR2、MMP9、TGF-β3表达水平上升与炎症因子IL-17表达水平上升呈正相关,差异具有统计学意义(P<0.05)。结论育龄子宫肌瘤患者子宫内膜组织炎症因子IL-17、雌激素受体激素水平以及纤维化因子存在高水平表达,且表达水平呈正相关趋势。     

Ossified soft tissue leiomyoma in a patient with sickle cell anemia

Osseous metaplasia in leiomyomas is extremely rare. Here, we report the case of an ossified subcutaneous leiomyoma in a 34-year-old African American man with sickle cell thalassemia who presented with a painful nodule of the scapular region, which appeared as a heavily mineralized soft tissue mass on chest radiographs. Histopathologic and immunohistochemical examination of the resected nodule revealed a benign soft tissue leiomyoma composed of intersecting fascicles of spindle cells that strongly expressed smooth muscle actin and caldesmon. Extensive intratumoral calcification and ossification were noticed. Only eight cases of ossified leiomyoma have been reported, of which two arose in the deep soft tissue.     

Palisaded and verocay body prominent leiomyoma of deep soft tissue

Leiomyoma of deep soft tissue is a very rare tumor, which is sometimes confused with leiomyosarcoma, histopathologically. We describe a case of palisaded and Verocay-body prominent leiomyoma of deep soft tissue. The lesion was characterized by areas of nuclear palisading with Verocay-body like formation in addition to the features of leiomyoma.     

A case of cutaneous symplastic leiomyoma - a rare variant of cutaneous pilar leiomyoma

We describe the case of a cutaneous symplastic leiomyoma in a 37-year-old woman who presented with a 4-year history of a painful slow growing lesion on the left upper arm. The lesion was excised and subjected to histological examination. A poorly circumscribed lesion was seen in the dermis composed of spindle shaped cells with marked nuclear pleomorphism. No mitotic figures or necrosis were seen. The cells stained strongly positive with desmin and smooth muscle actin, and negative with S100, melan A, MNF116 a mouse monoclonal antibody to cytokeratin and CK5/6. The diagnosis was felt to be in keeping with a cutaneous symplastic leiomyoma, a rarely reported variant of the pilar leiomyoma. Histologically, it shows features similar to the symplastic variant of uterine leiomyoma with cytological atypia, nuclear pleomorphism and minimal mitotic activity. Although the long-term outlook is probably benign, the presence of cytological atypia and mitoses in any spindle cell tumor is generally a concerning feature and warrants long-term follow up.     

Vascular Leiomyoma of the Scalp with a Small Deformity on the Skull Mimicking a Dermoid Cyst

Abstract: We report a vascular leiomyoma of the extracranial scalp with a small deformity on the skull in a child mimicking a dermoid cyst. The tumor was removed surgically, and histologic examination revealed a vascular leiomyoma. A small deformity‐like dimple on the skull improved without evidence of recurrence. The early surgical excision yielded good results.     

Multiple leiomyoma

A case of asymptomatic multiple leiomyoma along with its histopathological features is described.     

Cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma: a rare occurrence and potential diagnostic pitfall

A case of cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma is described. The patient was a 60‐year‐old man who presented with a painless nodule on his chest. Thorough clinical examination did not reveal any evidence of tumor elsewhere. A punch biopsy was performed which showed a benign spindle cell neoplasm with focal cellular pleomorphism that had smooth muscle differentiation and was clearly originating from the arrector pili muscle, consistent with a cutaneous symplastic pilar leiomyoma. Immunohistochemical studies were performed and the tumor cells were strongly positive for smooth muscle actin (SMA) and desmin. Given the clinical information of a mass on the chest, clinical evaluation was recommended and a re‐excision was performed. Histologically, the re‐excision showed two distinct populations of cells. The upper portion of the tumor mirrored the initial biopsy; however, the base of the tumor showed hypercellular areas composed of spindle cells with marked pleomorphism and increased number of mitoses. The diagnosis of a cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma was given. This is the first report of this association of such occurrence reported in the literature. Fons ME, Bachhuber T, Plaza JA. Cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma: a rare occurrence and potential diagnostic pitfall.     

A case of diffuse pilar leiomyoma or acquired smooth muscle hamartoma?

A 62-year-old woman presented with a skin-coloured indurated asymptomatic plaque, 150 mm in diameter on the knee. Histological analysis of a skin biopsy taken from the lesion showed haphazardly arranged bundles of smooth muscle in the deep dermis, characteristic of a pilar leiomyoma. Cutaneous pilar leiomyomas are rare, benign smooth muscle tumours arising from the arrector pili muscle, which usually appear as red or brown papules < 15 mm in diameter. This case is unusual in its clinical appearance and size, being at least 10 times larger than the largest previously documented cutaneous pilar leiomyoma. Similar lesions have also been previously reported under the term 'acquired smooth muscle hamartoma' (ASMH). These cases are also rare and have no known consistent clinical features. Histologically it is difficult to differentiate between ASMH and leiomyomas, and some authors would consider this case as a new report of ASMH. We feel, however, that as the lesion is composed of only one element and has grown out of proportion with the growth of the limb, it is more in keeping with a true tumour rather than a hamartoma. We suggest the term 'diffuse pilar leiomyoma' may be more appropriate in this case, representing a new clinical variant of cutaneous pilar leiomyoma.     

EPITHELIOID LEIOMYOMA: A CASE REPORT*

A rare benign skin tumour with the histology of an epithelioid leiomyoma is described in an eight-year-old boy. Some comments on malignant varieties are mentioned.