Dermatology Online Journal

Genital leiomyoma: Surgical excision for both diagnosis and treatment of a unilateral leiomyoma of the male nipple
Amor Khachemoune MD¹, Carlos Rodriguez², Stephen Lyle MD PhD³, and S Brian Jiang MD⁴
Dermatology Online Journal 11 (1): 20

1. Division of Dermatology, Georgetown University Medical Center, Washington, DC2. University of Illinois College of Medicine, Chicago, IL 3. Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School 4. Department of Dermatology, Beth Israel Deaconess Medical Center, Harvard Medical School

---

Abstract

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.

---

Clinical synopsis

Figure 1	Figure 2
Frontal view of both nipples (Fig. 1).
A 10- × 8-mm white to yellowish, hard papule is embedded in the left nipple (Fig. 2).

Figure 3	Figure 4
A poorly circumscribed dermal tumor exhibiting characteristic elongated, blunt-ended smooth-muscle nuclei and eosinophilic cytoplasm (H & E scanning magnification).
Smooth muscle fiber bundles interlace with variable amounts of collagen, appearing cigar-shaped (H & E 10 × magnification).

Figure 5	Figure 6
Positive staining with desmin (Fig. 5) (higher magnification).

A 61-year-old man presents with a 3-month history of an enlarging hard lesion on his left nipple. He reports that the small tumor grew progressively and became pruritic over the past 3 months; he denies any pain in response to cold, tactile, or emotional stimuli. He also states that there is no fluid drainage from this tumor, either with gentle pressure or spontaneously. His past medical history is significant for a prior prostatectomy to remove an adenocarcinoma, a procedure that was complicated by seminitis and bilateral joint infection with methicillin-resistant Staphylococcus aureus.

On physical examination, there is a 10- × 8-mm white to yellowish hard papule embedded in the left nipple (Figs. 1 and 2). There is no color change or eczematous reaction on the areola around the left nipple. The right nipple measures 4- × 4-mm. There is no axillary, supraclavicular, or inguinal lymphadenopathy. The remainder of his physical examination is unremarkable. A conservative excision of the nipple is performed. Histologic examination is consistent with a leiomyoma (Figs. 3, 4). The diagnosis is confirmed with desmin immunostaining (Figs. 5 and 6). On followup visit 10 days later, the patient states that the pruritus has completely disappeared. At his last visit 2 months after the procedure, he is still asymptomatic.

Discussion

Cutaneous leiomyomas are rare benign smooth muscle neoplasms that may easily evade clinical recognition [1]. These tumors are classified into four categories reflective of their respective sites of origin: (1) multiple piloleiomyomas, (2) solitary piloleiomyoma, (3) angioleiomyoma, and (4) genital leiomyoma [2]. The genital group includes masses arising from the dartos muscle of the scrotum or from the labia majora, as well as those derived from the mammilary muscle of the nipple in either sex.

Genital leiomyoma is the least commonly occurring type of cutaneous leiomyoma, and data to establish its various epidemiological tendencies are currently inadequate [2, 3]. The groin and nipple lesions are generally solitary asymptomatic masses, in contrast to their cutaneous counterparts, which are sometimes painful, either spontaneously or in response to cold, tactile, and emotional stimuli [1]. Nipple leiomyomas tend to be smaller than 2 cm in diameter, and must be clinically differentiated from angiolipomas, glomus tumors, eccrine spiradenomas, neurofibromas, nevi, lipomas, and breast carcinomas [1, 2]. The differential diagnosis also includes nipple adenomas, lymphocytoma benignum of Bafverstedt, and Paget's disease [4].

The diagnosis of nipple leiomyoma should be more earnestly entertained when complaints of pain are present without evidence of inflammatory changes [4, 5] This pain is thought to occur secondary to calcium-dependent contraction of smooth-muscle cells within the tumor [6] but may also be related to local pressure exerted on cutaneous nerves as well as the specific infiltrating cellular milieu [2].

Histologically, leiomyoma of the nipple appears as a poorly circumscribed dermal lesion exhibiting characteristic elongated, blunt-ended smooth muscle nuclei and eosinophilic cytoplasm. Smooth-muscle fiber bundles interlace with variable amounts of collagen, appearing cigar-shaped; on cross-section, cytoplasmic and perinuclear vacuolization become more evident and suggest smooth-muscle origin. Masson trichrome, aniline blue, van-Gieson, phosphotungstic acid-hematoxylin (PTAH), and desmin and actin immunohistochemical stains facilitate the histologic recognition of smooth muscle. Although multiple cutaneous leiomyomas can be associated with internal tumors [7], no known association exists for solitary genital leiomyoma, and thus no further workup for the patient is required.

An incisional biopsy or a conservative excision is indicated for tissue examination and pathologic diagnosis. Given the clinical presentation, we opted for a conservative excision after discussion with the patient.

Management of leiomyoma of the nipple depends primarily on the presence or absence of symptoms. Medical therapy plays a limited role, but calcium-channel blockers [2, 6] and α-adrenergic blockers [2] may help in palliating or eliminating associated pain through inhibition of smooth-muscle contraction. Gabapentin, a novel anticonvulsant with an uncertain mechanism of action, has also been used to control piloleiomyoma related pain [8, 9].

Surgical excision or ablation of a solitary mass, possibly including nipple, areola and subareolar ducts is another treatment option for painful lesions [2, 3], and CO₂-laser ablation has also been employed in cases of symptomatic cutaneous masses [2]. The prognosis for isolated lesions is excellent, particularly following surgical excision. However, recurrences have been reported following incomplete excision [5]. The possibility of recurrence is another reason to perform complete excision of the nipple. There is no documented tendency toward malignant degeneration in cutaneous leiomyomas.

In summary, this case is a rare example of leiomyoma presenting with pruritus of the nipple, with no associated pain; a conservative excision is both diagnostic and therapeutic.

References

1. Holst VA, Junkins-Hopkins JM, Elenitsas R. Cutaneous smooth muscle neoplasms: clinical features, histologic findings, and treatment options. J Am Acad Dermatol. 2002;46(2):477-490.

2. Miethke MC, Raugi GJ (January 21, 2003), Leiomyoma, [Online], eMedicine, Available from: http://www.emedicine.com/derm/topic217.htm [accessed April 10th, 2004].

3. Allison JG, Dodds HM. Leiomyoma of the male nipple. A case report and literature review. Am Surg. 1989;55(8):501-502.

4. Tsujioka K, Kashihara M, Imamura S. Cutaneous leiomyoma of the male nipple. Dermatologica. 1985;170(2):98-100.

5. Nascimento AG, Rosen PP, Karas M, Caron AG. Leiomyoma of the nipple. Am J Surg Pathol. 1979;3(2):151-154.

6. Thompson JA Jr. Therapy for painful cutaneous leiomyomas. J Am Acad Dermatol. 1985;13(5 Pt 2):865-867.

7. Martinez-Mir A, Glaser B, Chuang GS, Horev L, Waldman A, Engler DE, Gordon D, Spelman LJ, Hatzibougias I, Green J, Christiano AM, Zlotogorski A. Germline fumarate hydratase mutations in families with multiple cutaneous and uterine leiomyomata. J Invest Dermatol. 2003 Oct;121(4):741-4.

8. Scheinfeld N. The role of gabapentin in treating diseases with cutaneous manifestations and pain. Int J Dermatol. 2003 Jun;42(6):491-5.

9. Alam M, Rabinowitz AD, Engler DE. Gabapentin treatment of multiple piloleiomyoma-related pain. J Am Acad Dermatol. 2002 Feb;46(2 Suppl Case Reports):S27-9.

© 2005 Dermatology Online Journal