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.     

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.     

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.     

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.     

Multiple piloleiomyomas associated with solitary angioleiomyoma.

A 58 year-old male had reddish scattered papules strictly confined to the right side of his upper back and neck, right shoulder, and right upper arm. Additionally, he also noticed a subcutaneous nodule on his left thigh. Histopathological examinations revealed that the papules on the back, nape of the neck, shoulder, and arm were multiple piloleiomyomas, as shown by the proliferation of bundles of smooth muscles in the dermis. The subcutaneous nodule of the thigh was angioleiomyoma with a well-circumscribed lesion composed of smooth muscles and blood vessels. Ipsilaterality and segmentality of the distribution of the papules of piloleiomyomas and probable family history that his mother had similar papules with a similar distribution suggest the nevoid character of our case. Piloleiomyomas are often reported to be associated with leiomyoma of the uterus or other organs. This is the first reported case of multiple piloleiomyomas with solitary angioleiomyoma in the literature.     

Male genital leiomyomas showing androgen receptor expression

Genital leiomyoma in men include those superficial leiomyomas arising in the scrotum and the areola. They are unusual neoplasms: few cases have been reported in the literature and they usually escape clinical diagnosis. Three cases of male genital leiomyomas are reported: two in the scrotum and one in the areola. They were all conservatively excised and the behaviour was completely benign in all cases. Histopathological examination showed the typical findings of superficial leiomyomas, with some minor differences between cases arising in the scrotum and those from the areola. Immunohistochemical findings not only confirmed the smooth muscle nature of all cases but also showed unequivocal immunostaining for androgen receptors in the leiomyomas from the scrotum. Immunostaining for androgen receptors in scrotal leiomyomas is, as far as we are aware, a previously unknown characteristic of male genital leiomyomas. This finding supports the role of steroid hormones in the growth of genital leiomyomas, similar to leiomyomas found in other locations.     

S1-guideline cutaneous and subcutaneous leiomyosarcoma

Superficial leiomyosarcomas (LMS) are rare skin cancers (2–3% of cutaneous sarcomas) that originate from dermally located hair follicle muscles, dartos or areolar muscles (cutaneous/dermal LMS), or from vascular muscle cells of the subcutaneous adipose tissue (subcutaneous LMS). These superficial LMS are distinct from LMS of the deep soft tissues. Leiomyosarcomas are typically localized at the lower extremities, trunk or capillitium, and present as painful, erythematous to brownish nodules. Diagnosis is made by histopathology. The treatment of choice for primary LMS is complete (R0) microscopically controlled excision, with safety margins of 1 cm in dermal LMS, and 2 cm in subcutaneous LMS, if possible. Non-resectable or metastatic LMS require individual treatment decisions. After R0 resection with 1 cm safety margins, the local recurrence rate of dermal LMS is very low, and metastasis is very rare. Subcutaneous LMS, very large, or incompletely excised LMS recur and metastasize more frequently. For this reason, clinical follow-up examinations are recommended every six months for cutaneous LMS, and every three months for subcutaneous LMS within the first two years (in subcutaneous LMS including locoregional lymph node sonography). Imaging such as CT/MRI is indicated only in primary tumors with special features, recurrences, or already metastasized tumors.     

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.     

Eccrine angiomatous hamartoma: a report of symmetric and painful lesions of the wrists

Eccrine angiomatous hamartoma (EAH) is a rare, benign cutaneous lesion histologically defined as a proliferation of eccrine glands within a closely associated vascular stroma. Typically EAH presents as a solitary flesh-colored, hyperhidrotic, painful papule or plaque appearing at birth or during childhood. Only two previously reported cases have been described involving multiple, symmetrically located lesions. The occurrence of knuckle pads in patients with EAH has not been reported. We present an instance of multiple painful EAH lesions occurring symmetrically on the extensor wrists in a 14-year-old girl with knuckle pads.     

Leiomioma de la areola mamaria. Presentación de dos casos

—Cutaneous genital or dartoic leiomyomas are rare benign tumors originating in the smooth muscle fibers of the skin of the scrotum, vulva, nipple and areola. The latter are the less frequently observed.We report two cases of leiomyoma of the areola. Both presented as a solitary, small, nonulcerated nodule. Histopathological features shared similar characteristics: a non-encapsulated, ill-defined tumor in superficial and mid-dermis composed of spindle cells with elongated nuclei and eosinophilic cytoplasm, arranged in interdigitating fascicles. There were no atypia or mitoses. It was remarkable a reactive epidermal hyperplasia overlying the surface of the tumor, as is typically seen in the histiocytomas. Areolar leiomyoma is a benign lesion that is not known to undergo malignant transformation, although it may be confused clinically with carcinoma of the breast.     

Leiomyosarcoma of the lip

BACKGROUND: Superficial leiomyosarcomas are rare malignant tumors that may be subdivided into cutaneous and subcutaneous cases. CASE-REPORT: A 58 year-old man was seen for a lower lip tumor that appeared 3 years earlier. Histopathologic and immunohistochemical examinations showed features of cutaneous leiomyosarcoma. DISCUSSION: Cutaneous leiomyosarcoma may derive from the arrector pili, smooth muscle of sudoral glands or genital dartoic muscle. The subcutaneous form arises from smooth muscle wall of blood vessels. Superficial leiomyosarcomas occur frequently in the extensor surface of the lower extremities and involve the mucosal area in rare cases. Only five cases arising in the lips have been described. Lip cutaneous leiomyosarcoma may derive from ectopic sweat glands of the lips or from a hypodermic tumor that extends to the lip.     

Late-onset eccrine angiomatous hamartoma on the forehead

A 71-year-old Korean man presented with a solitary erythematous plaque on his forehead (Fig. 1). It was first noticed by the patient 1 year previously and had slowly increased in size over that time. Physical examination revealed a slightly elevated, 1.5 cm x 1.5 cm erythematous plaque on the upper midline of the forehead. Sweating was not evoked by physical work or emotional stress. There was no pain or tenderness associated with the lesion. The patient had no history of trauma to the forehead. Histopathologic examination of the lesion showed increased numbers of eccrine glands, as well as dilated vascular channels in the deep dermis and subcutaneous tissue (Fig. 2). An immunohistochemical study showed that these eccrine glands stained positively for S-100 and carcinoembryonic antigen (CEA), and the vascular channels for the antifactor VIII-related antigens. These findings are consistent with eccrine angiomatous hamartoma. There was no change in the lesion during the 1-year follow-up period.     

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.     

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.     

Genital porokeratosis: a series of 10 patients and review of the literature

BACKGROUND: Porokeratosis (PK) is an uncommon disorder of epidermal keratinization with a unique clinical appearance, unknown aetiology, and an unpredictable outcome. Genital PK (defined as localized PK confined to the genital area in this study) is extremely rare and is not well documented. OBJECTIVES: To evaluate the clinical manifestations, histopathology, clinical course and treatment response for genital PK. METHODS: We reviewed the clinicohistological data from 10 patients with genital PK seen at Chang Gung Memorial Hospital from 1990 to 2005. RESULTS: Seven patients had lesions only in the genital area, and three patients had genital and adjacent areas involved. All patients were male and the mean age at initial diagnosis was 46.0 years (range 36-59). All but one patient presented with pruritic lesions. Three patients (30%) had diabetes mellitus. No malignant transformation was observed. Surgical excision was the most effective treatment if it could be performed. CONCLUSIONS: Genital PK appeared more frequently in the Asian population than in reports from western countries. Genital PK presented mostly as pruritic lesions in Taiwan, with a wide age distribution. Long-term follow-up might be needed.     

Extragenital bowenoid papulosis associated with atypical human papillomavirus genotypes

BACKGROUND: Bowenoid papulosis typically appears as grouped violaceous or red-brown papules in the genital or perianal regions and clinically resembles condylomata acuminata. Isolated extragenital bowenoid papulosis is rare and has been reported in only a few case reports. OBJECTIVES: A 51-year-old immunocompetent, healthy woman had two solitary papules on the elbow; a 41-year-old HIV-positive man had a solitary cutaneous plaque on the abdomen. No genital, periungual, or other extragenital sites of involvement were noted in either patient. The diagnosis was confirmed histologically in both cases. Lesional skin from the female patient was tested with the Digenehybrid HPV DNA assay and was positive for a mixture of low-risk HPV subtypes (6, 11, 42, 43, 44). Lesional skin from the male patient was tested with polymerase chain reaction (PCR). Consensus primers targeted for the HPV L1 region, which is a highly conserved sequence common to more than 20 HPV subtypes encoding a viral capsid protein, were used. PCR using the consensus primers was positive, but type-specific probes for HPV types 6, 11, 16, 18, 45, 31, 33, 35, and 39 were negative. CONCLUSIONS: To our knowledge, our male patient represents the first case of isolated bowenoid papulosis of the abdominal skin. Isolated upper-extremity bowenoid papulosis in our female patient is also a unique case in both location and involvement of low-risk HPV types (6, 11, 42, 43, 44), which have not been previously associated with extragenital bowenoid papulosis.     

Metastasizing epithelioid hemangioendothelioma of the nose in childhood

Epithelioid hemangioendothelioma (EHE) arising in the skin is extremely rare, and the majority of documented cases have developed in soft tissues or parenchymatous organs. We report on a 9-year-old boy who presented with a painful erythematous plaque on the nose. Histopathological findings showed a dermal tumor composed of epithelioid cells with moderate cellular atypia and characteristic intracytoplasmic vacuoles. Immunohistochemical stainings confirmed the vascular nature of the tumor. On the basis of these findings, the diagnosis of an EHE was made. Then, two cervical lymph nodes were excised and adjuvant therapy with interferon alpha 2b was initiated. Eighteen months after diagnosis, there is no evidence of local tumor recurrence or metastases. To our knowledge, this is the first report of a metastasizing cutaneous EHE on the nose in childhood. As our case shows, this rare tumor entity has to be considered as a differential diagnosis in neoplasms of the skin, even in childhood.     

Specific cutaneous infiltrates of B-cell chronic lymphocytic leukemia (B-CLL) at sites typical for Borrelia burgdorferi infection

BACKGROUND: Cutaneous manifestations of B-cell chronic lymphocytic leukemia (B-CLL) comprise a wide spectrum of clinicopathologic presentations. In some cases, onset of skin lesions is triggered by antigenic stimulation, and specific skin infiltrates at sites of previous herpes simplex or herpes zoster infection have been well documented. Specific skin manifestations of B-CLL can also be observed at sites typical for lymphadenosis benigna cutis (nipple, scrotum, earlobe), a Borrelia burgdorferi-associated cutaneous B-cell pseudolymphoma. METHODS: We studied specific skin manifestations of B-CLL arising at sites typical for B. burgdorferi-induced lymphadenosis benigna cutis, analyzing tissues for presence of B. burgdorferi DNA using the polymerase chain reaction (PCR) technique. Six patients with B-CLL (M : F = 4 : 2; mean age: 67.8) presented with specific skin lesions located on the nipple (four cases) and scrotum (two cases). RESULTS: Clinically there were solitary erythematous plaques or nodules. Histology revealed in all cases a dense, monomorphous infiltrate of small lymphocytes showing an aberrant CD20+/CD43+ phenotype. In all cases monoclonality was demonstrated by PCR analysis of the JH gene rearrangement. PCR analysis showed in four of the six cases the presence of DNA sequences specific for B.burgdorferi. CONCLUSIONS: Our study demonstrates that infection with B. burgdorferi can trigger the development of specific cutaneous infiltrates in patients with B-CLL.     

Clinical, dermoscopic and histopathological evaluation of the Meyerson nevus: case report

Meyerson nevi occur whenever a rare focal and transitory eczematous eruption arises around melanocytic lesions. The same phenomenon has also been observed in non-melanocytic lesions as well. Herein we report the case of a 25 year old, male patient, who had noticed, two months before, the arising of a pruriginous and erithematous halo around two nevi localized on his abdomen. The lesions were found to be atypical on dermoscopic examination and he was submitted to surgical excision of both nevi. Histopathological examination revealed displastic compound melanocytic nevi, surrounded by intraepidermical vesicles and spongiosis. Present report suggests that Meyerson phenomenon does not seem to alter dermoscopic features of nevi.     

Cutaneous leiomyoma of the male nipple

A case of cutaneous leiomyoma of the male nipple is reported. This is the second case reported by dermatologists to the best of our knowledge.