CASE REPORT. A Rare Presentation of Liposarcoma of Spermatic cord: A Case report. Liposarcoma of Spermatic Cord

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1 CASE REPORT Dr. Mohammad Amjad Khan - Department of Urology, Deccan College of Medical Sciences & Owaisi Hospital & Research Centre, Dr. K. Eashwer Goud is Professor & Head, Department of Urology, Deccan College of Medical Sciences & Owaisi Hospital & Research Centre, Dr. Manjula Shantaram is Associate Professor of Biochemistry, Yenepoya Medical College, Yenepoya University, Mangalore, Karnataka, A Rare Presentation of Liposarcoma of Spermatic cord: A Case report Mohammad Amjad Khan, K. Eashwer Goud, Manjula Shantaram, Abdul Majeed, Adil Majid Paratesticular tumors represent 7-10% of intra scrotal masses. Sarcomas account for 90% of malignant lesions of the spermatic cord; of these approximately 3-7% are liposarcomas. They are most commonly found in the extremities, in the retroperitoneum and less often in the head and neck area. This report describes a patient with liposarcoma of the spermatic cord. The clinical presentation, pathogenesis, and prognosis of this unusual sarcoma is reviewed. The low-grade malignant potential and irregular growth characteristics of liposarcoma render preoperative diagnosis difficult. The role of radical orchiectomy, retroperitoneal lymph node dissection, radiation therapy and chemotherapy in the treatment of spermatic cord liposarcomas is discussed. Keywords: Liposarcoma, Radical orchiectomy, Radiation therapy, Chemotherapy. Dr. Abdul Majeed is Professor of Surgery, Yenepoya Medical College, Yenepoya University, Mangalore, Karnataka, Dr. Adil Majid is Associate Professor, Department of Urology, Deccan College of Medical Sciences & Owaisi Hospital & Research Centre, Corresponding Author: Dr. Mohammad Amjad Khan amjad_786_2000@yahoo.com October 2012 International Journal of Health and Rehabilitation Sciences Volume 1 Issue 2

2 INTRODUCTION Liposarcoma of the spermatic cord is a rare condition, representing approximately 7% of paratesticular sarcomas 1. Around 181 cases are reported in the world literature 2 among which half of the cases have been reported from Japan. There have been occasions when an effort had to be made to differentiate them from inguinal hernias, funicular cysts, lipomas of the cord, etc 3-6. Like in this case, preoperative diagnosis is not common and usually they present as operative or histological surprises. The recommended treatment is surgery in the form of wide local excision which in most cases turns out to be a radical high orchiectomy. There is no indication for routine lymph node dissections as the loco-regional lymph nodes are rarely involved. The outcome is fairly good in most cases if the resection is R0. Adjuvant radiotherapy is usually not required except in cases with positive margins or local recurrence and poor prognostic factors. There is no definite role of chemotherapy and most of the recommendations are based on cases with recurrences. These cancers are, however, known for local recurrences and long-term follow-up of up to 10 years is mandatory; as even recurrences after 20 years have been reported 5-7. CASE REPORT A 62 year old presented with the complaint of an insidiously progressing right inguinal swelling of 6 months duration. To begin with, the swelling was painless but developed pain in the few days prior to presentation. Cough aggravated the pain. He was hypertensive and diabetic and was on treatment for the same. He was also on treatment for tricuspid valve dysfunction post percutaneous transluminal coronary angioplasty. Clinical examination revealed a right inguinoscrotal swelling which measured around 8x 4cm. It was firm to hard in consistency. No cough impulse or fluctuation could be elicited. The swelling was irreducible and the trans-illumination test was negative. Right testis was distinctly felt separate from the swelling. There was no associated lymphadenopathy. The left inguinoscrotal region was normal. Abdominal examination did not reveal any other palpable lump. Systemic examination did not reveal any other Figure 1: Intra Operative Picture showing the Tumour abnormality. Routine investigations including chest x-ray were normal. Echocardiogram showed post angioplasty status. Ultrasound of the region revealed large right inguinoscrotal swelling incarcerated inguinal hernia or lipoma of the cord. Patient was planned for surgery wide local excision/ or right radical orchiectomy for the remote possibility of sarcoma under general anesthesia and appropriate consent was obtained. Through an inguinal approach, the spermatic cord was dissected and delivered out. It appeared as a firm to hard lipomatous mass (11x6cm) involving the entire circumference and was inseparable from the cord (Fig.1). The testis was also delivered into the wound and was found to be normal (Fig.2 & 3). An occlusion clamp was applied to the spermatic cord close to the deep ring and in view of the high suspicion of malignancy; a right radical high orchiectomy was done. The histopathological examination revealed dedifferentiated liposarcoma of the cord with clear margin of resection. The patient was not given any adjuvant treatment and he is on regular follow up from last 3 months without loco regional recurrence or progression of tumor. DISCUSSION The first case of a spermatic cord sarcoma was reported in 1845 by Lesauvage 8 and subsequently liposarcoma was first described in It is an uncommon neoplasm that accounts for 3-7% of paratesticular sarcomas 9. It grows slowly, and may reach 30 cm or more in size % of spermatic cord tumors are benign and usually originate from lipomatous tissue. This tumor is difficult to diagnose preoperatively and is often mistaken 108 October 2012 International Journal of Health and Rehabilitation Sciences Volume 1 Issue 2

3 Figure 2: Post-operative picture showing Tumor & Testis for incarcerated hernia, lipoma or hydrocele or so in our case also. The most common age of presentation is between years (range years). Regarding the origin of this cancer there are various theories, but most arise de novo. Simple lipomas that constitute nearly 80% of all neoplasm almost never transform into sarcomas 5-7,11,12. Various authors have hypothesized that a mesenchymal origin rather than malignant transformation of lipomatous cells leads to liposarcomas 7,11,12. Despite being a rare kind of tumor, liposarcomas of the spermatic cord presumably have good prognosis and a high 5-year survival rate 13. Due to the rarity of this condition, it is difficult to accumulate sufficient cases to learn its natural history and reach conclusions regarding treatment. For example, a 1981 series studied sarcomas of the spermatic cord at the Massachusetts Hospital. Only ten cases were collected between 1940 and 1977, and of these, two were liposarcomas 14. In a publication of Johns Hopkins Hospital, from 1980 to 2000 only three cases were identified 15. USG provides little information on paratesticular sarcomas, as some are visualized as homogenous and isoechogenic, others as inhomogeneous and echo-density being quite variable. The use of CT scans is not widely reported, but seems to be promising, as liposarcomas are of low density and can be well demarcated, but no pathognomonic features for the differentiation of benign versus malignant masses are defined 12. Use of MRI provides good information on the local extent, but an exact evaluation of any such mass again cannot be ascertained. FDG PET scan may be of use in Figure 3: Cut surface of the Tumor recurrent cases but their routine use is not indicated 16,17. The tumor is often classified histologically into 4 subtypes: Welldifferentiated, Myxoid and round cells, Pleomorphic, Dedifferentiated 18. Most paratesticular liposarcomas are welldifferentiated 19 (40-45%), sclerosing and inflammatory according to current criteria 9. This has a good correlation with the prognosis of these tumors, notwithstanding their large size according to Montgomery 15. For liposarcoma the most specific immunohistochemical marker is the S100 protein which is positive in up to 90% of cases. High-grade tumors are often positive for desmin. Liposarcomas are locally aggressive tumors and recurrence is quite common after incomplete excision. The inguinal radical orchiectomy with wide resection margin is the standard approach for sarcomas of the spermatic cord 20. Some authors favor a hemiscrotectomy in addition to the inguinal orchiectomy 21. Sometimes a second resection is advised if the margins are positive. Local radical excision alone seems to be insufficient for liposarcomas since local recurrence is a major problem occurring in up to 50% of the patients 21. Due to the rarity of this condition, the role of radiotherapy and chemotherapy has not yet been well established 9. A review by Stephen 22 in1995 reports the results of treatment in six cases of liposarcoma of the spermatic cord; the study shows that there is no response to radiotherapy and chemotherapy (doxorubicin). The article also mentions that retroperitoneal lymphadenectomy does not offer any therapeutic benefits 16,22. This is explained by the fact that the 109 October 2012 International Journal of Health and Rehabilitation Sciences Volume 1 Issue 2

4 main pattern of dissemination of sarcomas of the spermatic cord is by contiguity through the inguinal canal into the abdominal cavity; the blood and lymph routes are less common 20. In a study of all histological types of sarcomas of the spermatic cord, the 5-year survival rate was 75%, 50% of patients had recurrence and of patients with liposarcoma, only 4% had metastases at the time of diagnosis 7. A long periodic follow-up is recommended because the local control of this disease may be compromised by very late recurrences. These check-ups should include a chest x-ray and a bone scan (if there are suspicious bone symptoms) because although the incidence of metastatic dissemination via blood is low, it may still occur 16,23,24. CONCLUSION Sarcomas of the spermatic cord are rare neoplasms that should be considered in the differential diagnosis of scrotal masses. Imaging techniques such as scrotal ultrasound, axial tomography and magnetic resonance should be used to assess these masses. Initial management is surgical. We recommend even a transoperative assessment of the surgical edges in order to extend the margins of the excision if needed in order to try to reduce the chance of recurrence. Complementary treatment for recurrences is controversial as on date and must be assessed on an individual basis for each patient: for this reason we also recommend that these patients be followed-up closely for a longer period of time. REFERENCES 1. Sogani P, Grabstald H, and Whitmore Jr WF. Spermatic cord sarcoma in adults. Journal of Urology 1978; 120 (3): Malizia M, Brunocilla E, Bertaccini A, Palmieri F, Vitullo G, Martorana G. Liposarcoma of the spermatic cord: description of two clinical cases and review of the literature. Arch Ital Urol Androl 2005; 77 (2): Ushida H, Johnin K, Koizumi S, Okada Y. Liposarcoma of the spermatic cord in the left scrotum and inguinal region: a case report. Hinyokika Kiyo 2000; 46: Logan JE, Williams MB, Shaves ME, Mc Cammon KA. Liposarcoma of the spermatic cord: a case report and review of management. Uro Today Int J 2010, doi: /uij Hinman F, Gibson TE. Tumors of the epididymis, spermatic cord and testicular tunics: a review of literature and report of three new cases. Arch Surg 1924; 8: Demirci U, Buyukberber S, Cakir A, Ozturk B, Akyurek N, Unver B, Baykara M, Benekli M, Coskun U. Synchronous testicular liposarcoma and prostate adenocarcinoma: a case report. Cases J 2010; 3: Coleman J, Brennan MF, Alektiar K, Russo P. Adult spermatic cord sarcomas: management and results. Ann Surg Oncol 2003; 10: Hinman F and Gibson TE. Tumors of the epididymis, spermatic cord and testicular tunics: a review of literature and report of three new cases. Archives of Surgery 1924; 8: Fitzgerald S and MacLennan GT. Paratesticular Liposarcoma. J Urol 2009;181: Aránguez GM, Patiño EG, Fernández HC, Fernández AE. Liposarcomas paratesticular es. Estudio clinicopatológico de tres casos y revisión de la literatuta. Arch Esp Urol 2006; 59(9): Sogani PC, Grabstald H, Withmore WJ. Spermatic cord sarcoma in adults. J Urol 1978; 120: Cardenosa G, Papanicolaou N, Fung CY, et al. Spermatic cord sarcomas: sonographic and CT features. Urologic Radiology. 1990; 12(3): Martínez CP, Martínez CE, Martín AM, Zurita CJM, Pérez GM. Liposarcoma of spermatic cord. A rare urologic tumor. Arch Esp Urol 1992; 45(7): Blitzer PH, Dosoretz DE, Proppe KH, et al. Treatment of malignant tumors of the spermatic cord: a study of 10 cases and review of the literature. J Urol. 1981; 126: Montgomery E, Fisher C. Paratesticular Liposarcoma. Am J Surg Pathol 2003; 27(1): Goodman FR, Staunton MD, Rees HC. Liposarcoma of the spermatic cord. J R Soc Med 1991; 84: Lipset RE, Kirpekar M, Cooke KS, Abiri MM. US case of the day. Myxoid 110 October 2012 International Journal of Health and Rehabilitation Sciences Volume 1 Issue 2

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