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Year : 2017  |  Volume : 13  |  Issue : 2  |  Page : 276-278

Leiomyosarcoma of scrotum: A rare in-field second malignancy in a previously irradiated carcinoma of prostate and review of literature

Department of Radiation Oncology, Amrita School of Medicine, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham, Amrita University, Kochi, Kerala, India

Date of Web Publication23-Jun-2017

Correspondence Address:
Chelakkot G Prameela
Department of Radiation Oncology, Amrita School of Medicine, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham, Amrita University, Kochi - 682 041, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.189401

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 > Abstract 

Background: Curative intent treatments for malignancies using radiation therapy while achieving longer disease-free survivals, may also lead to solid second malignancies, a dreaded late complication. Both in-field as well as out-field second malignancies are encountered and lead to diagnostic dilemma, and delay in treatment.
Materials and Methods: Details of a case of rare, in-field, radiation induced leiomyosarcoma, a rare soft tissue sarcoma, in a treated case of high risk carcinoma of prostate, who presented to our tertiary care centre was retrieved and analysed.
Conclusion: Increasingly sophisticated radiotherapy techniques, while providing state of the art treatment options, does increase the potential risk of radiation-induced second-malignancies. Respecting integral-dose carries priority, until further studies elucidates mechanism of tumorigenesis.

Keywords: Leiomyosarcoma, radiation-induced sarcoma, radiation-induced second malignancies, soft tissue sarcoma

How to cite this article:
Prameela CG, Ravind R. Leiomyosarcoma of scrotum: A rare in-field second malignancy in a previously irradiated carcinoma of prostate and review of literature. J Can Res Ther 2017;13:276-8

How to cite this URL:
Prameela CG, Ravind R. Leiomyosarcoma of scrotum: A rare in-field second malignancy in a previously irradiated carcinoma of prostate and review of literature. J Can Res Ther [serial online] 2017 [cited 2022 Dec 2];13:276-8. Available from: https://www.cancerjournal.net/text.asp?2017/13/2/276/189401

 > Introduction Top

Soft tissue sarcomas (STS), arising from mesenchymal tissue, constitute <1% of all adult malignancies. Among STSs, leiomyosarcoma (LMS) accounts for 10%–20%.[1] Primary LMS of scrotum is rare. Solid second malignancies arising in irradiated field are documented, with higher incidence following treatment of childhood cancers,[2] and there are a few reports of radiation-induced sarcomas (RIS) following treatment of carcinoma cervix. Radiation-induced scrotal LMS, extremely rare, with only very few case reports, is highlighted here.

 > Case Report Top

A 77-year-old South-Indian was diagnosed to have high-risk carcinoma prostate, Stage-III (T3bN0M0-AJCC-7th edition) in 2007. He received neoadjuvant hormonal therapy using GnrH-analogue and anti-androgen medications, followed by 3-dimensional-conformal external-beam-radiotherapy (7000cGy/35 fractions) and was on long-term adjuvant hormonal therapy. He was on regular follow-up, with relevant investigations (imaging, serum prostate specific antigen).

Seven years later, in 2014, he presented with edema in groin and a gradually progressing left sided scrotal swelling with pain of 2 weeks duration. He denied history of trauma or hernia. Clinical examination revealed an area of black discoloration over the left scrotum. Ultrasonography showed well organized hetero-echoic collection with septations in the left scrotal sac.

Scrotal hematoma, benign cystic swelling of scrotum, and varicocele were considered as differentials and left scrotal exploration was done. Following evacuation of the scrotal hematoma peroperatively, a large proliferative mass was seen extending from perineum into left hemiscrotum, arising from prostate. Hard nodules were felt at the root of penis. Profuse bleeding ensued from the mass which was controlled with argon coagulator. Multiple biopsies were obtained from the mass, which was reported as pleomorphic LMS [Figure 1]a and [Figure 1]b. Three weeks postsurgery, patient presented with profuse bleeding from scrotal wound and severe pain [Figure 2]. Compression scrotal bandages were applied, and in view of persistent profuse bleeding, palliative hemostatic external-beam radiation (2000cGy/5-fractions) to the scrotal mass was offered [Figure 3]. He presented again after 4 weeks, with cough, exertional dyspnea, and bilateral pedal edema. Computerized tomogram of the chest revealed extensive bilateral pulmonary metastases [Figure 4]. Considering rapidly progressing disseminated disease, symptomatic best supportive care was offered. Patient succumbed to disease within a week of discharge from hospital.
Figure 1: (a) Histopathological image: Low power view: Showing features suggestive of Pleomorphic leiomyosarcoma. (b) Histopathological image: High power view (×40): Showing interlacing fascicles of spindle shaped cells with eosinophilic cytoplasm and cigar-shaped nuclei. Features suggestive of Pleomorphic leiomyosarcoma

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Figure 2: Clinical photograph taken at the time of radiotherapy planning: Showing oedematous scrotum with nonhealing wounds

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Figure 3: Computed tomography scan of pelvis done as a part of radiotherapy treatment planning: Axial view: Showing size and extent of scrotal growth

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Figure 4: Computed tomography scan of thorax: Axial view: Showing multiple bilateral pulmonary metastases

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 > Discussion Top

STS accounts for 1% of all cancers, and genitourinary tract STS constitute about 2.1% of this.[3] Subcutaneous LMS account for 1–2% of all superficial soft-tissue malignancies.[1] LMS arises from undifferentiated smooth muscle cells of mesenchymal origin. Johnson H Jr first reported LMS of scrotum in 1987. Scrotal LMS arises from paratesticular tissue or testicular parenchyma and paratesticular LMS from spermatic cord, scrotal tissues such as testicular-tunica, dartos muscle and scrotal-subcutis, or epididymis.

RIS are highly aggressive with an incidence of <0.2%.[4],[5] LMS as a RIS, in a patient treated with radical external-beam-radiotherapy, for primary prostatic carcinoma, is extremely rare with very few case reports in literature.[5] Neugut in 1997 first reported increased risk for second malignancy after radiation for prostate cancer, and Benner reported increased risk of in-field-sarcomas after prostatic irradiation. A recent review on risks of second malignancies following radiation therapy showed that one in seventy patients undergoing radiation to prostate and surviving >10 years can develop a second tumor.[6]

Arlen had modified Cohen's criteria postulated in 1948, for diagnosing RIS. Time interval between initial treatment and development of sarcoma is considered the most crucial factor. Sarcoma team of Memorial Sloan Kettering Cancer Centre suggested that six months latency period is sufficient to confirm diagnosis of RIS.[7] Latency period ranges from 3 to 36 years, with a median of 11 years, and has a bearing on histological type; shortest latency documented in liopsarcoma and longest in LMS.[7]

Criteria considered for diagnosing RIS are: (1) Patient should have received prior radiation (2) lesion should be located within the field of irradiation (3) histological features of second malignancy should be completely different compared to original lesion (4) latent period between radiation therapy and histological diagnosis of second malignancy, should be >4 years, and (5) patients with cancer syndromes, for example, Li-Fraumeni and Rothmund-Thomson should be excluded.[4]

RIS develop within the irradiated field receiving doses of 3000–6000 cGy.[8] Although definite dose-response-relation is not established, carcinomas develop in areas exposed to lower doses, while sarcomas arise in areas receiving higher dose. Lower and inhomogenous doses in the edge of treatment fields can cause genomic instability, and altered repair mechanism, which can progress to tumors. Stem-cells of sarcoma are believed to be dormant cells requiring very high doses of radiation to cause sufficient cell kill leading to proliferation and tumorigenesis. Wide arrays of molecular changes are implicated in the development of RIS, like alterations in RB1 and TP53 tumor-suppressor-genes and MYC-amplifications on chromosome 8q24. Change to intensity-modulated-radiotherapy while increasing risk of carcinomas has not much affected in-field RIS.[9]

Scrotal LMS present as painless, firm, irregular, rubbery, nontender masses without hydrocele, with radial, expansile growth pattern invading and infiltrating adjacent tissues;[1] and are often mistaken for benign lesions. Ultrasonogram or contrast enhanced computed tomography scan or both, helps in assessing the extent of lesion. Chest imaging completes metastatic workup. Histopathological examination of tissue biopsy is confirmatory. Spindle cells with cigar-shaped nuclei arranged in inter-weaving-fascicles are characteristic [Figure 1]a and [Figure 1]b.[1]

Surgery remains the standard-of-care, and wide-local-excision with adequate margin is offered, though an oncologically safe margin is not clearly defined. McKee showed improved 5-year local recurrence free interval with margin >10 mm (84%), suggesting that adequate negative margin translates to superior local control.[10]

The role of chemotherapy is controversial, be it in neoadjuvant or adjuvant setting. R0 resection with good margin whether requires adjuvant chemotherapy remains vague. Metastatic and recurrent lesions are considered for combination chemotherapy regimens.[4] Palliative chemotherapy is considered in a select group refusing surgery. There is no consensus on role of radiation therapy and is usually offered as a palliative approach.[1]

The overall prognosis of patients with LMS is poor with estimated median survival of 17 months.[11] Metastatic disease at presentation and positive surgical margins portend to worse survival. RIS tend to have poorer disease specific survival than sporadic STSs.[4],[7]

 > Conclusion Top

With increasing availability of sophisticated radiation treatment options, awareness of potential risk of development of RIS, even after decades, is critical, as salvage options can be offered with early diagnosis. Respecting integral-dose carries priority until further studies elucidate mechanism of tumorigenesis.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Batra A, Marwah N, Marwah S, Gupta S, Sen R. Subcutaneous leiomyosarcoma of scrotum presenting as an exophytic mass: An unusual presentation. Indian Dermatol Online J 2015;6:193-5.  Back to cited text no. 1
[PUBMED]  [Full text]  
Berrington de Gonzalez A, Gilbert E, Curtis R, Inskip P, Kleinerman R, Morton L, et al. Second solid cancers after radiation therapy: A systematic review of the epidemiologic studies of the radiation dose-response relationship. Int J Radiat Oncol Biol Phys 2013;86:224-33.  Back to cited text no. 2
Haran S, Balakrishan V, Neerhut G. A rare case of paratesticular leiomyosarcoma. Case Rep Urol 2014;2014:715395.  Back to cited text no. 3
Horiguchi H, Takada K, Kamihara Y, Ibata S, Iyama S, Sato T, et al. Radiation-induced leiomyosarcoma of the prostate after brachytherapy for prostatic adenocarcinoma. Case Rep Oncol 2014;7:565-70.  Back to cited text no. 4
Dalton DP, Rushovich AM, Victor TA, Larson R. Leiomyosarcoma of the scrotum in a man who had received scrotal irradiation as a child. J Urol 1988;139:136-8.  Back to cited text no. 5
Sountoulides P, Koletsas N, Kikidakis D, Paschalidis K, Sofikitis N. Secondary malignancies following radiotherapy for prostate cancer. Ther Adv Urol 2010;2:119-25.  Back to cited text no. 6
Gladdy RA, Qin LX, Moraco N, Edgar MA, Antonescu CR, Alektiar KM, et al. Do radiation-associated soft tissue sarcomas have the same prognosis as sporadic soft tissue sarcomas? J Clin Oncol 2010;28:2064-9.  Back to cited text no. 7
Ruben JD, Davis S, Evans C, Jones P, Gagliardi F, Haynes M, et al. The effect of intensity-modulated radiotherapy on radiation-induced second malignancies. Int J Radiat Oncol Biol Phys 2008;70:1530-6.  Back to cited text no. 8
Hall EJ, Wuu CS. Radiation-induced second cancers: The impact of 3D-CRT and IMRT. Int J Radiat Oncol Biol Phys 2003;56:83-8.  Back to cited text no. 9
McKee MD, Liu DF, Brooks JJ, Gibbs JF, Driscoll DL, Kraybill WG. The prognostic significance of margin width for extremity and trunk sarcoma. J Surg Oncol 2004;85:68-76.  Back to cited text no. 10
Fisher C, Goldblum JR, Epstein JI, Montgomery E. Leiomyosarcoma of the paratesticular region: A clinicopathologic study. Am J Surg Pathol 2001;25:1143-9.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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