| Abstract|| |
Soft tissue sarcomas of the genitourinary tract are rare. Paratesticular sarcomas are extremely rare with majority of scrotal masses localizing to the testis and being neoplastic in nature. Paratesticular leiomyosarcomas (LMSs) are located in the spermatic cord, epididymis, or scrotum. However, their location in the scrotal skin or subcutaneous tissue is extremely rare. Only 10 cases have been reported from India previously. Ours is the 11th case. A 50-year-old male presented with a recurrent scrotal mass which was painless and gradually increasing in size. Histopathology and immunohistochemistry confirmed it to be paratesticular LMS. A rare case report with the review of literature is presented.
Keywords: Leiomyosarcoma, recurrent, scrotum
|How to cite this article:|
Agrawal R, Gupta M, Mohan N, Sharan J, Kumar P. Recurrent leiomyosarcoma scrotum: An important differential in scrotal masses. Indian J Pathol Microbiol 2017;60:581-3
|How to cite this URL:|
Agrawal R, Gupta M, Mohan N, Sharan J, Kumar P. Recurrent leiomyosarcoma scrotum: An important differential in scrotal masses. Indian J Pathol Microbiol [serial online] 2017 [cited 2020 Feb 22];60:581-3. Available from: http://www.ijpmonline.org/text.asp?2017/60/4/581/222978
| Introduction|| |
Majority of all paratesticular leiomyosarcomas (LMS) are located in the spermatic cord or epididymis. Their location in the scrotal skin or subcutaneous tissue is exceptionally rare. These tumors usually present as slow-growing firm, irregular, rubbery, nontender masses and are confirmed by histopathology.
| Case Report|| |
A 50-year-old male presented with swelling over left-sided scrotum along with a nonhealing ulcer for 2 months. According to the patient, he was apparently asymptomatic 2 months back when he noticed a painless mass in the scrotum, which was initially smaller and then started increasing in size. He has been a known case of diabetes mellitus. He also gave a history of being operated twice for the same condition first time 10 years back and second time 3 years back. He did not have any record of the previous surgeries. On examination, there was an ulcerated mass present over the scrotum with rolled up edges and hypertrophied floor [Figure 1]a. The growth was not fixed to the testis or underlying structures. It bled on touch. Serum lactate dehydrogenase, beta-human chorionic gonadotropin, and alpha-fetoprotein levels were within normal limits. The mass was excised in total. Grossly, a partially skin-covered soft tissue piece along with hair measuring 3 cm × 4 cm × 3.5 cm was received. Microscopy showed intervening fascicles of malignant spindle-shaped cells [Figure 1]b. Nuclei were large, elongated, hyperchromatic with irregularly clumped chromatin and blunt ends (cigar-shaped). Cytoplasm was moderate in amount and eosinophilic [Figure 1]c. Mitotic figures (4–5/10 high power field [HPF]) and few multinucleated giant cells were present. No areas of hemorrhage, necrosis, or hyaline change were noted. Immunohistochemistry showed positivity for desmin [Figure 2]a and vimentin [Figure 2]b; whereas CD 34 [Figure 2]c, S-100 [Figure 2]d, and Van Gieson stains [Figure 1]d were negative. As per the FNCLCC grading system, tumor differentiation – Score 1, mitotic count – Score 1, and tumor necrosis – Score 0, i.e., total score of 2 was found in the case; thus, the histological features were consistent with a diagnosis of Grade I LMS. Chest X-ray and computed tomography of the abdomen and pelvis did not reveal any evidence of metastasis. The patient was advised for regular long-term follow-up and currently has been disease-free for 18 months following surgery.
|Figure 1: (a) Gross photograph of the scrotal mass. (b) Photomicrograph showing interlacing bundles of neoplastic spindle cells (H and E, ×100). (c) Photomicrograph showing hyperchromatic nuclei with blunt and eosinophilic cytoplasm (H and E, ×400). (d) Photomicrograph showing negative staining with van Gieson (H and E, ×100)|
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|Figure 2: (a) Immunohistochemistry stains showing (a) desmin positivity (×100), (b) vimentin positivity (×100), (c) CD34 negativity (×100), (d) S-100 negativity (×100)|
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| Discussion|| |
Johnson in 1987 reported the first known case of LMS of the scrotum. It has been suggested that the rarity of LMSs of the scrotum is such that a General Practitioner would usually see such a tumor once every 20 years. Soft tissue sarcomas account for 1% of all malignancies. LMSs constitute 10%–20% of soft tissue sarcomas., Genitourinary LMSs arise in the urinary bladder, kidney, or prostate and constitute about 2.1% of soft tissue sarcomas. LMSs of the scrotum, not involving the testis, epididymis, or spermatic cord, are rare and belong to the group of subcutaneous superficial LMSs., Paratesticular LMSs originate from testicular tunica (48%), spermatic cord (48%), epididymis (2%), and dartos muscle and scrotal subcutaneous tissue (2%). Extensive literature search revealed <40 cases of scrotal LMS and only 10 cases from India.,,
It is mostly diagnosed in the sixth decade, and more than 80% of patients are over 40 years old., They usually present as firm, rubbery, nontender, irregular masses that tend to be slow-growing and evolve over years. The size of tumor is usually between 2 and 9 cm. It is often mistaken for a benign lesion. Local irradiation is a possible etiologic factor.
Microscopy reveals spindle cells with cigar-shaped nuclei arranged in interweaving fascicles. The diagnosis of malignancy in LMS is based on the mitotic rate of 2–10 mitoses/HPF; in addition to this, the presence of nuclear pleomorphism, vascular invasion, tumor depth, infiltration and the percentage of tissue necrosis are also considered. FNCLCC grading system is recommended for these tumors and includes tumor differentiation (Score 1–3), mitotic count (Score 1–3), and tumor necrosis (Score 0–2). The total score of 2, 3 gives a Grade I, score of 4, 5 gives a Grade 2, and scores of 6–8 gives a Grade 3 tumor. LMSs are subdivided topographically into three groups: LMS of the deep soft tissue, LMS of the cutaneous and subcutaneous tissue, and LMS of vascular origin. Paratesticular LMS originates from the spermatic cord, the scrotum (testicular tunica, dartos muscle, and scrotal subcutis), or the epididymis. The most common spermatic cord type arises from undifferentiated mesenchymal cells of the cremasteric muscles, vas deferens, and arterial walls. The epididymal form is less frequent and arises from the smooth muscles surrounding the basement membrane of the epididymis canal. Scrotal types arise from the dartos layer. On immunohistochemistry, LMSs are positive for actin and desmin.
Other rare tumors, including dermatofibrosarcoma protuberans (DFSP), benign leiomyoma, fibrous mesothelioma, various benign fibrous tumors and pseudotumors, and fibromatosis, should be considered in the differential diagnosis of paratesticular LMSs. DFSP was ruled out due to less cytological atypia, less mitotic figures, and CD34 negativity in the present case.
Lymphatic spread may involve the external iliac, hypogastric, common iliac, and retroperitoneal lymph nodes while hematogenous metastases are primarily pulmonary.The vas deferens can act as a conduit allowing local spread to the scrotum, inguinal canal, or pelvis. LMS lacks a surrounding capsule so that a complete excision with a surgical clear margin is usually not possible. These grow by radial expansion, infiltrating the local tissues as they proliferate. Owing to the small number of patients in the literature, definitive data regarding the role of adjuvant therapy are limited. They may play a role in abrogating the tumor's hematogenous metastatic potential. Inguinal lymph node dissection is not advocated unless a high degree of suspicion of metastasis is present. Late local recurrence and distant metastases occur in some cases, so long-term follow-up is recommended. Prognosis of LMS depends on the site, size, depth, and histopathological grade of the tumor and the presence or absence of distant metastases., The presence of mitotic activity, percentage of necrosis, and severity of nuclear pleomorphism are all evaluated to grade the disease. Immunohistochemistry reveals positivity for actin and desmin. CD34 and cytokeratin expression have also been reported in some cases. It has been observed that 30% of the patients had recurrence, 30% had metastases (lymph nodes, lungs, liver), and 30% died after 4-year follow-up. A surgically positive margin detected at the first excision dramatically increases the risk of local recurrence.
| Conclusion|| |
LMS should be considered as a differential diagnosis in any elderly male presenting with intrascrotal mass. Adjuvant radiation therapy can lead to improved locoregional control with a role in patients with nonmetastatic paratesticular LMS or in those who refuse surgery.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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Dr. Ranjan Agrawal
Department of Pathology, Rohilkhand Medical College Hospital, Pilibhit Bypass Road, Bareilly, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]