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LETTER TO EDITOR Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 3  |  Page : 447-448
Primary leiomyosarcoma of the penis


1 Department of Pathology, Cancer Institute (W.I.A.), 38, Sardar Patel Road, Chennai - 600 036, India
2 Department of Surgical Oncology, Cancer Institute (W.I.A.), 38, Sardar Patel Road, Chennai - 600 036, India

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Date of Web Publication12-Aug-2009
 

How to cite this article:
Sundersingh S, Majhi U, Narayanaswamy K, Balasubramanian S. Primary leiomyosarcoma of the penis. Indian J Pathol Microbiol 2009;52:447-8

How to cite this URL:
Sundersingh S, Majhi U, Narayanaswamy K, Balasubramanian S. Primary leiomyosarcoma of the penis. Indian J Pathol Microbiol [serial online] 2009 [cited 2014 Nov 27];52:447-8. Available from: http://www.ijpmonline.org/text.asp?2009/52/3/447/55028


Sir,

We report a rare case of deep-seated leiomyosarcoma of the penis in a gentleman who underwent total penectomy for a tumor involving the distal shaft and glans penis. Sarcomas of the penis are very uncommon representing <5% of all types of malignant tumors occurring at this site. Leiomyosarcoma is the second most common sarcoma of the penis following Kaposi sarcoma. [1]

A 56-year-old male presented with pain and swelling of the penis of one and a half months duration. On examination, there was a 2 x 2 cm indurated ulcer with raised edges and hard plaque-like thickening involving the distal shaft of the penis on the right side and dorsal aspect extending onto the glans penis. No inguinal or iliac nodes were palpable. Ultrasound of abdomen and pelvis were normal. Computed tomography scan of chest was normal. Wedge biopsy from the lesion showed a tumor composed of fascicles of spindle cells with eosinophilic cytoplasm and hyperchromatic elongated cigar-shaped nuclei with blunt ends exhibiting moderate to marked nuclear atypia. Mitoses were 0-2/hpf [Figure 1]. Immunohistochemistry showed positivity of the tumor cells for vimentin, muscle actin, desmin and smooth muscle actin [Figure 2]. Stain for keratin was negative. Proliferative activity assessed by Ki-67 was 40%. A diagnosis of leiomyosarcoma Grade 3 was made. Patient underwent total penectomy. Gross examination showed a 2 x 2 cm ulcerated tumor involving the dorsal aspect of the right distal shaft of penis extending onto the glans penis. Cut surface showed a gray-white, firm, deep-seated tumor measuring 3.5 x 3 x 3 cm. Histopathological examination showed features suggestive of leiomyosarcoma Grade 3 involving the corpora cavernosa and corpus spongiosum [Figure 3]. Urethral mucosa was free. Areas of necrosis and degeneration were seen. Resected margins were free.

Leiomyosarcoma is the second most common sarcoma of the penis. Leiomyosarcoma of the penis has been classified into superficial and deep subtypes depending on the site of origin. [2] They can arise from 1) the dartos muscle of the prepuce and shaft of penis 2) the erector pilorum muscle of the penile shaft 3) the muscle wall of superficial vessels and 4) the smooth muscle of the deep vessels that constitute the corpora cavernosa and corpus spongiosum. Tumors arising from the former three sites constitute the superficial type and the last site, the deep type.

Deep-seated tumors present as large, rapidly growing poorly circumscribed firm masses with a tendency to invade urethra. [3] On gross examination, they are usually rubbery in consistency with white or gray cut surface. Microscopically they are composed of interlacing fascicles of spindle cells with blunt-ended cigar- shaped nuclei. Nuclear atypia varies from mild to severe and mitoses ranges from 2-14/10hpf. Areas of necrosis may be present. Immunohistochemistry shows positivity for vimentin, muscle-specific actin, smooth muscle actin and desmin.

The most common differential diagnosis includes Kaposi sarcoma characterized by slit-like spaces with numerous red cells, intracytoplasmic PAS-positive, diastase-resistant hyaline globules and a prominent lymphoplasmacytic infiltrate. Kaposi sarcoma exhibits strong immunoreactivity for CD31 and CD34 and negative reactivity for desmin. Malignant fibrous histiocytoma rarely arises in the penis. It exhibits a prominent storiform- pleomorphic pattern and shows negative reactivity for actin and desmin. Leiomyosarcoma is distinguished from sarcomatoid carcinoma of the penis by the lack of cellular atypia of the surface epithelium and absence of immunoreactivity for keratin.

Surgery remains the mainstay of treatment. Small deep-seated tumors located in the distal shaft or glans penis may be effectively treated by partial penectomy while large deep-seated tumors, particularly those situated at the root of the penis require total penectomy. [2]

Regional lymph node dissection is usually not indicated as nodal metastases are uncommon. [4] Adjuvant chemotherapy and radiotherapy have no clear role in the management of penile leiomyosarcoma. However, since the deep type is associated with recurrence and distant metastases, combination of local pelvic radiotherapy and systemic chemotherapy might be effective as an adjuvant therapy. [5]

Tumor depth and size seem to be the best predictors of clinical outcome with deep-seated large tumors often associated with a poor prognosis. [2] Histological prognostic parameters include tumor growth pattern (circumscribed or infiltrative), high mitotic count (> 10 mitoses/10 hpf) and Grade 3 histology. [2] Our patient is alive and on follow-up, six months following surgery.

In conclusion, we report an additional case of deep-seated leiomyosarcoma of the penis with the histological differential diagnoses and treatment options for this rare entity.

 
   References Top

1.Fetsch JF, Miettinen M. Tumors of the penis. In: Eble JN, Sauter G, Ebstein JI, Sesterhenn IA, editors. Pathology and genetics of tumors of the urinary system and male genital organs. Lyon: IARC Press; 2004. p. 292-6.  Back to cited text no. 1    
2.Fetsch JF, Davis Jr CJ, Miettinen M, Sesterhenn IA. Leiomyosarcoma of the penis: a0 clinicopathologic study of 14 cases with review of the literature and discussion of the differential diagnosis. Am J Surg Pathol 2004;28:115-25.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Dominici A, Delle Rose A, Stomaci N, Pugliese L, Posti A, Nesi G. A rare case of leiomyosarcoma of the penis with a reappraisal of the literature. Int J Urol 2004;11:440-4.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Pow-Sang MR, Orihuela E. Leiomyosarcoma of the penis. J Urol 1994;151:1643-5.  Back to cited text no. 4  [PUBMED]  
5.Nanri M, Kondo T, Okuda H, Tanabe K, Toma H. A case of leiomyosarcoma of the penis. Int J Urol 2006;13:655-8.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]

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Correspondence Address:
Shirley Sundersingh
New No.23, Jambulingam Street, Nungambakkam, Chennai - 600 034
India
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DOI: 10.4103/0377-4929.55028

PMID: 19679995

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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