Testicular leiomyosarcoma with metastasis.

Primary leiomyosarcoma of testis is a rare entity with few cases reported in literature. Primary leiomyosarcoma of testis usually occurs following radiotherapy or long-term anabolic steroid use. Without these predisposing factors, its occurrence is rare. In the present study, we present a rare case of primary leiomyosarcoma of testis occurring in an elderly patient, with an unusual presentation mimicking epididymo-orchitis and metastasis eight months following high inguinal orchidectomy.

INTRODUCTION

Primary testicular leiomyosarcoma is very rare with only a few cases being reported in the literature till date. They should be differentiated from the more common sarcomas of the spermatic cord as well as from germ cell tumors. There is lack of data on the natural history, histological criteria for diagnosis and treatment recommendations because of rarity of this disease. Most cases reported in the literature indicate that this may be an indolent tumor with a potential for cure if treated early.

CASE REPORT

A 55-year-old man without any history of radiotherapy or anabolic steroid intake presented with history of left testicular swelling associated with pain and fever of one and half month duration. He was treated elsewhere with antibiotics for two weeks with a diagnosis of left epididymo-orchitis. Physical examination revealed an 8 × 5 cm hard mass in the left testis. The spermatic cord was normal. Ultrasound of the scrotum showed a 6 × 5 cm hypoechoic mass lesion in left testis without any calcifications, suggestive of a tumor. The contralateral testis was normal. Computed tomography (CT) scans of abdomen and chest were unremarkable. Tumor markers, alpha-fetoprotein and beta-human chorionic gonadotropin, were within normal limits. The patient underwent left high inguinal orchidectomy. The cut section of the testis showed an intraparenchymal, grey-white, poorly encapsulated lesion measuring about 7 × 5 cm, pushing the normal parenchyma to one pole. Epididymis was normal. Microscopy [Figure 1] showed a tumor composed of interlacing fascicles and whorls of elongated spindle cells with eosinophilic cytoplasm and hyperchromatic nucleus with mitosis (8/10 hpf). Epididymis was uninvolved. Immunohistochemistry was positive for desmin. These findings were consistent with the diagnosis of a primary high-grade intratesticular leiomyosarcoma.

Figure 1

High power photomicrograph (×400) showing testicular parenchyma being compressed (arrow) by tumour tissue composed of interlacing fascicles and whorls of elongated spindle cells.

Postoperatively, the patient did not receive any adjuvant therapy, as there was no evidence of nodal or distant metastasis. He presented eight months later with diffuse soft tissue and bone metastasis and was referred for chemotherapy [Figure 2]. He received Adriamycin single-agent chemotherapy—the standard regimen for advanced or metastatic soft tissue sarcomas from other sites—but succumbed after two cycles, within three months of diagnosis of metastasis.

Figure 2

Patient presented with diffuse soft tissue and bone metastasis (arrows) eight months after high inguinal orchidectomy.

DISCUSSION

Primary sarcoma of the testis is a rare entity with diagnosis being made only after exclusion of more common paratesticular smooth muscle tumors.[1] Leiomyosarcoma arises from undifferentiated smooth muscle cells of mesenchymal origin.[2] Scrotal leiomyosarcomas can arise from either paratesticular tissue or the testicular parenchyma itself. Intratesticular leiomyosarcoma occurs from smooth muscle cells within the testis such as blood vessels, seminiferous tubules, and tunica. Its occurrence has been associated with radiotherapy and long term anabolic steroid use.[3] These tumors have a low metastatic potential[2] and can coexist with germ cell tumors which have an adverse prognosis.[4]

Before diagnosing it as primary leiomyosarcoma, a careful effort must be made to look for the presence of germ cell elements within the tumor.[4] Histological criteria for the diagnosis of intratesticular leiomyosarcoma are lacking in literature. In the presence of significant nuclear atypia, a mitotic count ≥10/10 high-power fields and coagulative necrosis with nuclear debris are criteria for the diagnosis of uterine spindle-cell leiomyosarcoma.[2] The same diagnostic criteria may well be used for the diagnosis of intratesticular leiomyosarcoma,[2] with subsequent immunohistochemical profiling to support the diagnosis of a primary intratesticular smooth muscle tumor.[1]

Primary intratesticular leiomyosarcoma usually occurs in fourth and fifth decades. Ultrasound of the scrotum usually shows a well-circumscribed, hypoechoic mass lesion with or without calcification. CT scans of the abdomen and chest are required to rule out metastasis. High inguinal orchidectomy is the treatment of choice, which should be followed by surveillance.[1] The role of radiotherapy and chemotherapy are not known. The few cases described in the literature do not support any adjuvant therapy.[5] Contrary to this finding, our patient developed diffuse metastases within a short period after treatment of the primary tumor and was subsequently referred for chemotherapy. Compared with leiomyosarcomas from other sites, prognosis with testicular leiomyosarcoma appears to be better. Till date, only ten cases of primary testicular leiomyosarcoma have been reported.[245] Of these reported cases, one died of pulmonary metastasis.[2]

Sarcoma of the testis usually presents as a painless swelling. However in our case, the patient presented with features suggestive of epididymo-orchitis, which is an uncommon presentation. Most cases described were of low grade, except for our case and the one described by Kumar et al.[2] The finding that patients with high-grade tumors presented with distant metastases shortly after treatment of the primary lesion suggests that it may be preferable to advise adjuvant chemotherapy in these cases. Moreover, patients with low-grade tumors can present with late metastasis, and hence close surveillance is recommended.[2]

Adjuvant chemotherapy with single-agent adriamycin or adriamycin plus ifosfamide has been the mainstay of treatment for advanced and metastatic soft tissue sarcomas from other sites.[6] The same regimens can be used as palliative therapy for testicular leiomyosarcomas. On this premise, our patient received Adriamycin, but the outcome was poor. Rarity of the condition and hence, lack of literature makes it difficult to define and optimize therapy.