Disseminated cutaneous leiomyomas

Cutaneous leiomyomas are benign smooth muscle tumors. Depending on the site of origin, one distinguishes three different types--piloleiomyoma, angioleiomyoma and genital leiomyoma. They appear between the first and third decades of life. A 56-year-old woman presented with painful red - brown papules and nodules on her trunk. Based on clinical and histological criteria the diagnosis of disseminated cutaneous leiomyomas was made. Because of the widespread cutaneous involvement, surgical treatment was not possible. Therefore we decided to employ a pharmacologic anti-depressive treatment. With this approach, the patient experienced considerable pain reduction.     

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.     

Bilateral Multisegmental Zosteriform Leiomyoma Cutis: A Rare Entity

Cutaneous leiomyomas are benign tumors derived from the smooth muscles of the skin. They are firm, skin-colored to brownish, frequently painful papules and nodules presenting either as a solitary tumor or multiple clustered lesions. Uncommon patterns of multiple leiomyoma cutis include linear, zosteriform, or dermatomal-like arrangement of lesions. We saw a 32-year-old man who presented with clusters of skin colored to reddish brown, occasionally painful papules and nodules distributed segmentally over C6 to T8 dermatomes on left side and T11 to L1 dermatomes on right side. Histopathology confirmed the diagnosis of cutaneous leiomyoma. The case is reported here for the occurrence of this benign cutaneous neoplasm in an extremely rare bilateral multisegmental distribution.     

Zosteriform pilar leiomyoma.

Pilar leiomyomas are usually multiple and are commonly found on the extensor aspects of the arm, trunk, and face. They are rarely distributed in a linear fashion or seem to follow a dermatome. We report a case of painful, unilateral, zosteriform cutaneous leiomyoma in a 25-year-old housewife. Histopathology revealed interlacing smooth muscle bundles arising from arrector pili muscles. Nifedipine (10 mg) twice daily was remarkably effective in diminishing the pain in our patient.     

Treatment of three hereditary leiomyomatosis patients with cryotherapy

Hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome is an autosomal dominant disorder characterized by cutaneous leiomyomas (CLM), uterine leiomyomas, and the increased risk of renal cell carcinoma. Piloleiomyomas develop from the arrectorpili muscle and are usually painful. For 22% of the affected patients, the pain is reported to impair their life quality. Since there are few case reports about cryotherapy for cutaneous leiomyomas in the literature, we have decided to present three patients who had painful cutaneous leiomyomas treated with cryotherapy.     

Multiple painful leiomyomas of the skin: a novel therapy with sympathicolysis?

Leiomyomatosis cutis is a rare disease involving large areas of the body surface with painful leiomyomas. Only a small panel of drugs has provided limited effects in the management of paroxysmal pain attacks associated with these tumours. Two patients with multiple, otherwise therapy-refractors cutaneous leiomyomas predominantly on the extremities underwert experimental transient sympathicolysis. Experimental cold exposure and noradrenulin injections resulted in increasing pain and vasoconstriction. whereas sympathicolysis counteracted this with pain reduction and vasodilatation. Our results confirm that α-adrenergic fibres play an important role in smooth muscle contraction and pain induction in cutaneous leiomyomas. Sympathicolysis should be induced in the therapeutic choices available for multiple painful leiomyomas localized on the extremities.     

Familial cutaneous and uterine leiomyomas: case report

Cutaneous leiomyomas are rare, benign tumors arising from the arrectores pilorum muscles of the skin, the tunica dartos of the scrotum, muscles of the areola of the nipple, and vulvar or vascular smooth muscles. Multiple cutaneous leiomyomas originate from the arrectores pilorum muscles of the skin (piloleiomyomata cutis). Occasionally, they seem hereditary and may be associated with uterine myomas. We present a family in which the mother and 4 of her 6 daughters had uterine myomas. All sisters had to undergo hysterectomy before the age of 40, and three of them had multiple cutaneous leiomyomas simultaneously. Our observations support the suggestion that this kind of leiomyomas is a disorder with autosomal dominant inheritance with incomplete gene penetration. Moreover, the data indicate the necessity of periodical examinations to rule out the presence of uterine myomas not only in cutaneous leiomyoma patients, but also in other women in a given family.     

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 type 2 segmental manifestation in a family with cutaneous leiomyomatosis

Two types of segmental manifestation have been differentiated in autosomal dominant skin disorders. Type 1 reflects heterozygosity for the underlying mutation and the severity of lesions is similar to that observed in the nonmosaic phenotype. Type 2 reflects loss of heterozygosity and the segmental involvement is characterized by a more pronounced manifestation superimposed on the ordinary phenotype. We here describe an unusual familial occurrence of type 2 segmental manifestation of cutaneous leiomyomatosis. The proposita, a 37-year-old woman with a history of multiple uterine leiomyoma, showed multiple agminated firm, pale-red papules arranged in an 8 x 20 cm triangular area on her right chest. A few similar isolated lesions were found to be scattered all over her body. Her 70-year-old mother reported likewise a history of multiple uterine myoma and showed an accumulation of leiomyomas in a band-like area extending from her left chest to the extensor surface of her left forearm. Some isolated nonsegmental lesions were found in other areas of her body. In 1955, one lesion had been surgically removed and histopathological examination showed features of leiomyoma. Moreover, the family history revealed cutaneous leimyomatosis in the proposita's grandmother and in one aunt. Most likely, this familial occurrence of a segmental type 2 manifestation of cutaneous leiomyomatosis in two generations resulted from postzygotic loss of heterozygosity at an early developmental stage. We hypothesize that the underlying gene locus is particularly prone to mitotic recombination or other postzygotic mutational events resulting in loss of the corresponding wild-type allele.     

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.     

Hereditary leiomyomatosis and renal cell carcinoma syndrome: a case report

The simultaneous occurrence of multiple cutaneous leiomyomas, uterine leiomyomatosis, and renal cancer is described as a cancer syndrome with an autosomal dominant pattern of inheritance. We report a 79-year-old man who presented with multiple hyperkeratotic, erythematous nodules on his right leg with a histological diagnosis of pilar leiomyoma. In a review of systems, gross hematuria, weight loss, and bone pain were noted. His pathologic diagnosis was determined to be metastatic papillary renal cell carcinoma. A family history revealed that his sister had a hysterectomy for uterine leiomyomas. The findings in this case can be attributed to hereditary leiomyomatosis and renal cell carcinoma syndrome.     

Leiomyoma cutis: a clinicopathological series of 37 cases

Background:

Cutaneous leiomyomas are benign smooth muscle tumors that comprise three distinct types such as piloleimyoma, angioleiomyoma, and genital leiomyoma.

Aim:

The objective of this study was to report a series of cases seen in last 8 years in a tertiary care hospital in north India and to discuss their clinicopathologic findings.

Material and Methods:

Paraffin-embedded blocks of cases reported as cutaneous leiomyoma from 1999 to 2007 were retrieved from the Institute of Pathology, New Delhi, and their clinical parameters were noted. Their histopathological features were reviewed on hematoxylin-eosin stained slides. Immunohistochemistry was performed where necessary.

Results:

Twenty-seven cases of piloleiomyoma, three cases of angioleiomyoma, five breast leiomyomas, and two scrotal leiomyomas were seen in patients ranging from 21 to 65 years of age, with an average of 38.2 years at presentation. There was a male predominance with 26 males and 11 females (M:F = 2.2:1). Solitary lesions (n = 21) were more common than multiple ( n = 16). The trunk and upper limbs were involved most commonly, comprising 23 of 37 (62.2%) cases. This was followed by lower limb, face, breast, and scrotum.

Conclusion:

Cutaneous leiomyomas are rare lesions and form an important clinical differential diagnosis of painful papulonodules. These must be biopsied in order to differentiate them from other spindle cell lesions.     

Cutaneous leiomyomas lack estrogen and progesterone receptor immunoreactivity

Gonadotropin-releasing hormone analog therapy is useful in treating uterine and some extrauterine smooth muscle tumors. These smooth muscle tumors have been demonstrated to have estrogen receptor and progesterone receptor immunoreactivity. The estrogen receptor and progesterone receptor immunoreactivity of smooth muscle tumors of the skin has not been reported. We evaluated 15 examples of cutaneous leiomyomas for estrogen receptor and progesterone receptor with ER-1D5 antibody and PGR-1A6 antibody. None of the 15 cutaneous leiomyomas demonstrated positive staining by this method. The tumorigenesis of cutaneous leiomyomas does not appear to be related to estrogen or progesterone receptor-mediated effects.     

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.     

Atypical pilar leiomyomatosis: an unusual presentation of multiple atypical cutaneous leiomyomas

Cutaneous leiomyomas are relatively common benign smooth muscle tumors that may arise as solitary or multiple lesions. Rare forms with cytologic atypia, and features similar to symplastic leiomyomas of the uterus, have been described. We report a case of multiple cutaneous atypical leiomyomas occurring in a 43‐year‐old man with long history of lesions of the right lower leg and a family history of leiomyomatosis. Twenty of the lesions were excised due to pain and were examined histopathologically. All the lesions exhibited features described in atypical leiomyomas of the skin including increased cellularity, nuclear atypia and pleomorphism, and low mitotic activity. The biologic potential of cutaneous atypical leiomyomas is uncertain. Only a few case reports exist in the literature with the majority occurring as solitary lesions. Most of the reported atypical leiomyomas have behaved in a benign fashion. However, a rare account of transformation to leiomyosarcoma emphasizes the need for long‐term follow up of these patients. Herein, we describe a case of multiple atypical cutaneous leiomyomas arising in the setting of familial leiomyomatosis.     

Multiple cutaneous and uterine leiomyomatosis (Reed's syndrome)

A 51-year-old woman with a history of uterine fibroids status post myomectomy and hysterectomy presented for evaluation and treatment of intermittently painful papules of the left shoulder. Histopathologic examination showed a proliferation of smooth muscle fascicles consistent with the diagnosis of cutaneous leiomyomas. Genetic sequencing demonstrated a novel mutation in the fumarate hydratase gene that confirmed the diagnosis of Reed's syndrome. A subset of individuals with Reed's syndrome is predisposed to develop a papillary renal-cell carcinoma, so appropriate radiologic examinations should be performed. Treatment of the pain caused by cutaneous leiomyomas includes the use of nifedipine, nitroglycerine, phenoxybenzamine, surgical excision, and carbon dioxide laser ablation.     

Scrotal leiomyoma

Genital leiomyomas of the scrotal skin are extremely rare benign tumors, originating from the tunica dartos of the scrotum. We describe a 55-year-old caucasian male with a 3-year history of a slowly growing asymptomatic scrotal knot which was first clinically regarded as a cyst and finally diagnosed histopathologically as leiomyoma. This typical case demonstrates that leiomyomas should be considered in the differential diagnosis of scrotal tumors.     

硝本地平缓解多发性皮肤平滑肌瘤引起的剧烈疼痛1例

报道一例伴剧烈疼痛的多发性皮肤平滑肌瘤患者，用硝苯地平缓解其疼痛。患者女，52岁，皮肤多发性结节性皮损，组织病理证实为平滑肌瘤。经硝苯地平10mg3次/日治疗后，疼痛明显缓解。     

Multiple familial cutaneous leiomyoma

INTRODUCTION: Cutaneous leiomyoma is a benign tumor, the discovery of which may suggest a hereditary form. We report a family in which 5 generations developed cutaneous and uterine leiomyomas. The originality of this report lies in the large number of generations developing the disease and the association with chronic myeloid leukemia. OBSERVATIONS: We have studied 16 members of a family with cutaneous and uterine leiomyomas spanning five generations. Eight members of the family (6 women and 2 men) presented with cutaneous leiomyomas. All 6 women also had uterine myomas with complications (menometrorrhagia, miscarriage, premature delivery and hysterectomy). Pathological association was also confirmed: polycythemia (1 case), papillary renal carcinoma (1 case) and chronic myeloid leukemia (1 case). DISCUSSION: Piloleiomyoma can develop sporadically or can be transmitted genetically. To our knowledge, we report the fifth case of a family of more than 2 generations presenting with piloleiomyoma. By studying the family tree, we were able to confirm the dominant autosomal nature of the mode of transmission found by other authors. The association of piloleiomyoma and uterine myoma is classified as Reed's syndrome. In such cases, the uterine myoma requires particularly careful monitoring since it is associated with significant risk of gynecological complications (menometrorrhagia, miscarriage, premature delivery and postpartum hemorrhage). Moreover, in our observations we describe diseases associated with piloleiomyoma: polycythemia (1 case), papillary renal carcinoma (1 case), but also the association of piloleiomyoma with chronic myeloid leukemia (1 case). A previous report described the same genetic deletion in uterine myoma as in chronic myeloid leukemia, which gives further weight to this association.     

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.