We present a case of cutaneous leiomyomas (CL) arising in a pleomorphic adenoma (PA) of the parotid gland. PA and CL are benign tumors arising from the parotid gland and the erector pilli muscle, respectively. They both have a benign clinical course and in most cases leiomyomas are multiple in nature. PAs of the parotid are the most frequent benign tumors of the major salivary glands. To our knowledge this is the first case of PA with CL.
Cutaneous leiomyomas are benign tumors originating from the erector pilli muscles. They have equal distribution in both sexes, (1) a benign clinical course, and most often present as multiple cutaneous lesions. Nevertheless, some cases have been described in which single cutaneous leiomyomas appear and even cases in which they appear in families. (1) There are reports that associate cutaneous leiomyomatosis to tumors located in other organs, specifically in the uterus (2) and kidneys. (3) Pleomorphic adenomas of the parotid are the most frequently found benign tumors of the major salivary glands (4) and their simultaneous appearance with other neoplasms has not been reported. We found no cases of patients with cutaneous leiomyomatosis and pleomorphic adenoma of the parotid gland after a thorough search of the English literature. Moreover, no genetic relation has been found between parotid tumors and leiomyomas.
A 38-year-old man presented with a 20-year history of multiple non-mobile confluent subcutaneous nodules of varying sizes that appeared like very large erythematous patches in some areas. The affected areas were the right side of the face, neck, and back of the trunk (Figure 1). The nodules were extremely painful and the pain was aggravated by clothing touching his skin. The patient also presented with a 1-year history of 2 cm nodules in the left inframandibular region. These lesions were firm and adhered to the deep planes, and appeared to be originating from the parotid gland (Figure 2). Multiple skin biopsies of the lesions were performed and routine histopathology revealed a well-demarcated nodule of spindle-shaped cells with cigar-shaped nuclei and acidophilic cytoplasm which were laid in bundles (Figure 3). The diagnosis of cutaneous leiomyomas was made. A fine needle aspiration biopsy (FNAB) of the parotid tumor was performed and a chondromyxoid background was encountered, as well as some small plasmacytoid cubic and cylindrical cells, consistent with pleomorphic adenoma (Figure 4a and 4b). The tumor was resected through a left superficial parotidectomy with an "S" technique. The facial nerve was identified and preserved and there was no manipulation of the deep parotid lobe. The 2 leiomyomatous plaques responsible for most of the patient's pain, on the right mandibular edge, were removed and the area was reconstructed with an advancement flap in the dissection plane. The lesion at the middle section of the neck was resected, creating a wound of approximately 10 X 9 cm, which was closed with a bilobed flap of the posterior base. The cephalic flap was of the myocutaneous type, with the platysma included. The secondary lobe was used to close the donor defect of the main flap and the donor defect of the secondary flap was closed primarily by widely dissecting the supraclavicular area. The remaining cutaneous defect was closed with a full thickness skin graft taken from the left supraclavicular area. The patient tolerated the procedure well and the post-operative recuperation was satisfactory with no complications (Figure 5). At the time of publication there has not been a recurrence of PA or CL, although there are remaining plaques and nodules in areas of where most of the CL was excised. No further complications have been reported.
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Smooth muscle neoplasms can be distributed throughout the entire body. The dermis contains smooth muscle fibers which are located in the erector pilli muscles, blood vessel walls, and the dartos muscle of the scrotum, vulva, nipple, and areola. There are three types of smooth muscle neoplasms: leiomyosarcomas, leiomyomas, and smooth muscle hamartomas (Becker's Nevus). In general, smooth muscle hamartomas are rare congenital lesions that run in families. They present in the trunk or in the limbs as lightly pigmented patches. The histopathology is characterized by bands of smooth (non-striated) muscle haphazardly oriented in the dermis.
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Leiomyosarcomas are malignant tumors which are divided into dermal leiomyosarcomas, subcutaneous leiomyosarcomas, and secondary tumors. They prefer the extensor areas of the limbs and occasionally cause pain. Unlike leiomyomas, leiomyosarcomas have poorly defined edges and its cells show atypia and pleomorphism. It is possible to observe at least one mitosis in 10 high-power fields. (5)
Leiomyomas may measure from a few millimeters up to several centimeters. Clinically, they present as reddish brown tumors, most frequently found on extensor surfaces, face, and neck. Histologically, they are well-circumscribed neoplasms made up of spindle-shaped cells with cigar-shaped fine chromatin nuclei and abundant eosinophilic cytoplasm. Leiomyomas are located in the dermis and may reach the subcutis; they are generally divided into 3 categories: 1) Solitary or multiple, originating from the erector pilli muscle (piloleiomyomas); 2) Angioleiomyomas, which are thought to arise from vascular smooth muscle; and 3) leiomyomas, originating from the dartos muscle of the genitalia, areola, and the nipple. Multiple cutaneous leiomyomas appear in young adults and are characterized by the development of a large number of tumors that may range from hundreds to thousands.
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In 1995, Fernandez et al reported a dominant autosomal transmission with variable penetrance. (1) However, other works have found normal karyotypes in patients presenting multiple leiomyomas with no other associated lesions. (6) Cutaneous leiomyomatosis may be associated to tumors of the uterus and kidney. Cases related to uterine myomas may appear in a syndrome of multiple uterine and cutaneous leiomyomas. This syndrome is linked to an alteration in a locus of chromosome 1q42.3-43 (2) and an association to locus 1q was observed in cases related to renal cancer. (7) Pain is the cause for most patients seeking medical attention and surgical excision is the treatment of choice. (1,8) Multiple treatments have been used to mitigate pain, such as nitroglycerin, phenoxybenzamine, or nifedipine. However, these treatments do not always yield good results and they are contraindicated in some patients, which is why the C[O.sub.2] laser has also been used with some benefit. (9)
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Pleomorphic adenomas most frequently appear in women in their fifth decade of life. (10) They are the most common tumor of the salivary glands in children and adolescents (11) and present as slow-growing, asymptomatic tumors. In a study of different types of tumors of the salivary glands, it was found that pleomorphic adenomas showed a common alteration in chromosomes 9q12-q21.11 and 16q11.2. (12) Macroscopically, they appear as well-defined nodules with a capsule that may be absent. Histologically, they may present a wide spectrum of patterns, nevertheless, in most tumors there is a mixture of mesenchymal and epithelial elements with a myxoid matrix containing epithelial cells. (13) The treatment of choice is a total or superficial parotidectomy. (14, 15) This case of cutaneous leiomyomatosis and parotid tumor is presented because of the associations reported between cutaneous leiomyomas and other neoplasms. We performed an extensive literature review and found no associations, genetic or otherwise, between CL and PA.
The clinical association in this patient may be coincidental. Nevertheless, clinicians must be aware of the possible association of leiomyomatosis and other neoplasms.
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Address for Correspondence
Jorge Ocampo-Candiani MD
Madero y Gonzalitos
Monterrey Nuevo Leon
Mexico, C.P. 64460
Phone: 52 (81) 83 481 465
Fax: 52 (81) 83 484 407
Jorge Ocampo-Candiani MD, (a) Osvaldo Vazquez-Martinez MD, (a) Arturo Regalado-Briz MD, (b) Oralia Barboza-Quintana MD, (c) Nora Mendez-Olvera MD (c)
a. Dermatology Department
b. Plastic and Reconstructive Surgery
c. Anatomic Pathology and Cytopathology Department University Hospital of the U.A.N.L, Monterrey, Nuevo Leon, Mexico
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