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Primary Sarcoma of the Prostate and Thrombocythemia in Adult; A First Case Report in English Literature
Case Reports
PRIMARY SARCOMA OF THE PROSTATE AND THROMBOCYTHEMIA IN ADULT; A FIRST CASE REPORT IN ENGLISH LITERATURE Puneet Gupta*, Shikha Roy **, Om Prakash Singh** , SK Rawat**** and Deepshikha Arora# *Senior Consultant, **Resident, Medical Oncology, ***Senior Consultant Urology, #Senior Pathologist, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India. Correspondence to:Dr. Puneet Gupta, Senior Consultant, Medical Oncology, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110076, India. Sarcoma is an unusual type of prostate cancer in adult. Prostate sarcoma (PS) is rare with only 183 cases reported in the English language medical literature from 1938-1998. Here we present a case of african.national who presented with locally advanced prostate sarcoma with thrombocythemia. However responded favourably to his initial trearment. He was treated with anthracycline based systemic chemotherapy; monoclonal antibody /large molecular target therapy (cyclophosphamide, liposomal adriamycin, vincristine,Cetuximab) for first three months without grade 3 or 4 toxicity. Then given synchronous chemo-target-radiation therapy in view of favourable initial radiological response and clinical benefit (effective palliation in terms of pain control and bleeding control). Key words: Prostate sarcoma, Adult, Treatment response.
CASE A 38 years aged Tanzanian man presented in April of 2008 with complaints of hematuria and difficulty in passing urine since Sept-Oct 07. Patient has had asthma since childhood. His father had died of prostate cancer. The physical examination was within normal limits excepts tenderness in lower abdomen and occasional rhonchi.Transrectal ultrasound (TRUS) done in February had shown enlargement of prostate and initially it was thought that he has had BPH. In view of continuation of his problems , in the same month he underwent cystoscopy and biopsy of prostatic mass in Tanzania . A histopathological diagnosis of adenocarcinoma was achieved through cystoscopic biopsy However at his arrival at Apollo the young patient presented with severe pain (score 9/10); frequent blockage of Foley’s urinary catheter; frank hematuria and depressive mood. Patient had no significant omorbid conditions. He has been not pretreated with any anti cancer therapy. His blood investigations showed thrombocythemia.His bone marrow examination was unremarkable.The chest X-ray and bonescan were normal The pelvic MRI showed large pelvic mass in the region of the prostate and urinary bladder base involving the right posterolateral wall of the urinary bladder and had a large intravesical component.There was loss of fat planes with both seminal vesicles,the right obturator internus muscle and the distal rectum and anal canal.Afew small nodes were seen along the right internal iliac vessels.Whole body PET scan Apollo Medicine, Vol. 5, No. 3, September 2008
234
showed increased FDG uptake in peripheral region of the mass.No other site of abnormal FDG uptake detected. The diagnosis of Malignant mesenchymal tumour with myogenic differentiation;Pleomorphic rhabdomyosarcoma was confirmed through histopathological study.of cystoscopic biopsy including immunohistochemistry (Fig.1). The rarity of case and need for newer drug were discussed with family. In view of advanced unresectable disease he was being treated with systemic chemotherapy along with target therapy. He received 3 cycles of chemotherapy (cyclophosphamide, liposomal adriamycin, VCR) on a 4 weekly schedule,given a loading dose of cetuximab (375 mg/m2) and then weekly 200 mg per sqm from May 08 to Aug 08. His repeat MRI and done in, July 08 revealed localized disease with clinical benefit in terms of effective pain (score 2/5) and bleeding control.However in view of continued unresectable status patient started on synchronous chemo-target therapy. DISCUSSION Prostate sarcoma is rare with only 183 cases reported in the English language medical literature from 1938-1998 [1-3]. Prostate sarcomas reach a much larger size before causing symptoms whereas sarcomas of the urinary bladder cause symptoms at smaller size and earlier stage [1]. Most patients with sarcoma of the prostate have symptoms related to bladder outlet obstruction. In 103 informative cases, 89 % of patients reported symptoms related to urinary obstruction. In 18% of cases, symptoms related to rectal
Case Report
S-2767/08
PASD
Vimentin
CK
PSA
235
Apollo Medicine, Vol. 5, No. 3, September 2008
Case Report
Desmin
MyoD1
PTAH
obstruction were observed. Major symptoms and characteristics of the prostate on palpation in prostate sarcoma in these 103 patients include 91 (89%) related with bladder outlet obstruction; 19 (18%) related to rectal obstruction; 6 (6%) pain; 4 (4%) hematuria; 1 (1) weight loss.
Fig. 1.
Morphology and immunohistochemistry for Pleomorphic Rhabdomyosarcoma of the prostate.
Apollo Medicine, Vol. 5, No. 3, September 2008
Characterization palpation (33 patients) include 21 (65%) smooth surface; 2 (6%) globular; 10 (29%) Irregular. In 40 patients 8 (21%) had soft or boggy consistency; 6 (16%) elastic of tense; 26 (63%) firm or hard [4]. This patient presented with features related to urinary and bowel obstruction and underwent suprapubic cystectomy. Diagnosis in usually made by needle biopsy and using immunohistochemistry as in this case. Prostate sarcoma may show an irregular hypoechoic prostate man with an anechoic area by ultrasonography [5-7]. A study involving these patients with RMS revealed that local extent of the disease could be delineated at the time of trans rectal needle
236
Case Report
biopsy [6]. Generally most prostate sarcoma are locally extensive but not metabolic at the time of diagnosis among 20 patients, 4 (20 %) showed metastatic disease at the time of diagnosis. The organ sites of metastases. The organ sites of metastases were long ( 2 cases [7,8] )], bone (2 cases [8,9]), and liver (1 case [3] ). In the absence of evidence of metastatic disease, aggressive local surgical excision should be performed. Most prostate sarcomas are usually quite large at the time of diagnosis. For locally extensive sarcomas involving the rectum, a total pelvic exenteration is indicated. Bilateral pelvic lymphadenectomy along with cysto prostatectomy is usually performed for respectable disease [1]. However this patient was locally advanced and unresectable throughout.
with adult prostatic sarcoma in world literature; also Pet Scan findings in p RMS and clinical benefit by chemo-target therapy has been likely described for first time in English literature. REFERENCE 1. Nicholas J Vogelzang, Peter T Scardino, William U Shipley, Frans MJ Debruyne, W Marsten Linehan. Comprehensive text book of genito urinary oncology. Chapter-55 (third edition ) 2005 Lippincott Williams & Wilkins. 2. Froehner M, Lossnitzer A, Manseck A, Koch R, Noack B, Wirth MP. Favorable long-term outcome in adult genitourinary low-grade sarcoma. Urology. 2000; 56(3):373-377. 3. Baker ME, Silverman PM, Korobkin M.Computed tomography of prostatic and bladder rhabdomyosarcomas. J Comput Assist Tomogr. 1985; 9(4): 780783.
Patient with low grade leiomyosarcomas with positive resection margins should receive post operative radiation therapy to lower the risk of local recurrence. Patients with high grade leiomyosarcomas or rhabdosarcomas and those with nodal metastases should generally receive adjuvant chemotherapy. A combination chemotherapy require that includes doxorubicin, seems most advisable, despite no clear evidence of benefit [1]. In the report of one of the largest single institution experiences, among six case treated with neoadjuvant chemotherapy or chemo radio therapy, three achieved size reduction of the primary tumor [10]. Leiomyosarcomas of the prostate (p LMS) have a better prognosis then prostatic rhabdomyosarcoma (p RMS) , with a 5 years survival of 42% for 53 cases of p LMS in contrast to a 13% 5 year survival rate for 21 cases of p RMS. No obvious improvement in survival rates for patients with PS was identified through more than 50 years [11]. Two reports of the largest single institution experience have revealed the existence of occasional long term survivors [10]. Negative surgical margin and absence of metastatic disease at presentation have been reported as predictive of long term survival [10]. The 5 years survival for prostate sarcomas was 28% out of total 97 patients, all of 21 patients with pRMS prostate died within 8 years. While a few survived out of 53 pLMS-P after 10 years [1]. This patient is undergoing treatment and intention is effective palliation. CONCLUSION This may be the first reported case of persistent thrombocytosis ,as an paraneoplastic syndrome associated
4. Dekernion JB, Paulson DF eds. Genitourinary cancer management, Herr HW. Sarcoma of the urinary tract. Philadelphia, PA: Lea and Fibger, 1987; 259-270. 5. Terris MK Transrectal ultrasound appearance of radiation-induced prostatic sarcoma Prostate.1998; 37:182-186. 6. Terris MK, Eigner EB, Briggs EM, Reese JH, Torti FM, Freiha FS. Transrectal ultrasound in the evaluation of rhabdomyosarcoma involving the prostate.Br J Urol. 1994; 74(3):341-344. 7. Yao JC, Wang WC, Tseng HH, Hwang WS.Primary rhabdomyosarcoma of the prostate. Diagnosis by needle biopsy and immunocytochemistry. Acta Cytol. 1988; 32(4): 509-512. 8. Bos SD, Slaa ET. An adult man with a rhabdomyosarcoma of the prostate. A case report.Scand J Urol Nephrol. 1991; 25(4): 329-330. 9. Waring PM, Newland RC. Prostatic embryonal rhabdomyosarcoma in adults: a clinicopathologic review Cancer 1992; 69: 755-762. 10. Sexton WJ, Lance RE, Reyes AO, Pisters PWT, Tu Shi-ming Pisters LL. Adult prostate sarcoma: The M.D. Anderson Cancer Center Experience. J Urology. 2001;166: 521-525. 11. Cheville JC, Dundore PA, Nascimento AG, Meneses M, Kleer E, Farrow GM, Bostwick DG. Leiomyosarcoma of the prostate. Report of 23 cases. Cancer. 1995; 76(8):1422-1427.
237
Apollo Medicine, Vol. 5, No. 3, September 2008
PRIMARY SARCOMA OF THE PROSTATE AND THROMBOCYTHEMIA IN ADULT; A FIRST CASE REPORT IN ENGLISH LITERATURE Puneet Gupta*, Shikha Roy **, Om Prakash Singh** , SK Rawat**** and Deepshikha Arora# *Senior Consultant, **Resident, Medical Oncology, ***Senior Consultant Urology, #Senior Pathologist, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India. Correspondence to:Dr. Puneet Gupta, Senior Consultant, Medical Oncology, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110076, India. Sarcoma is an unusual type of prostate cancer in adult. Prostate sarcoma (PS) is rare with only 183 cases reported in the English language medical literature from 1938-1998. Here we present a case of african.national who presented with locally advanced prostate sarcoma with thrombocythemia. However responded favourably to his initial trearment. He was treated with anthracycline based systemic chemotherapy; monoclonal antibody /large molecular target therapy (cyclophosphamide, liposomal adriamycin, vincristine,Cetuximab) for first three months without grade 3 or 4 toxicity. Then given synchronous chemo-target-radiation therapy in view of favourable initial radiological response and clinical benefit (effective palliation in terms of pain control and bleeding control). Key words: Prostate sarcoma, Adult, Treatment response.
CASE A 38 years aged Tanzanian man presented in April of 2008 with complaints of hematuria and difficulty in passing urine since Sept-Oct 07. Patient has had asthma since childhood. His father had died of prostate cancer. The physical examination was within normal limits excepts tenderness in lower abdomen and occasional rhonchi.Transrectal ultrasound (TRUS) done in February had shown enlargement of prostate and initially it was thought that he has had BPH. In view of continuation of his problems , in the same month he underwent cystoscopy and biopsy of prostatic mass in Tanzania . A histopathological diagnosis of adenocarcinoma was achieved through cystoscopic biopsy However at his arrival at Apollo the young patient presented with severe pain (score 9/10); frequent blockage of Foley’s urinary catheter; frank hematuria and depressive mood. Patient had no significant omorbid conditions. He has been not pretreated with any anti cancer therapy. His blood investigations showed thrombocythemia.His bone marrow examination was unremarkable.The chest X-ray and bonescan were normal The pelvic MRI showed large pelvic mass in the region of the prostate and urinary bladder base involving the right posterolateral wall of the urinary bladder and had a large intravesical component.There was loss of fat planes with both seminal vesicles,the right obturator internus muscle and the distal rectum and anal canal.Afew small nodes were seen along the right internal iliac vessels.Whole body PET scan Apollo Medicine, Vol. 5, No. 3, September 2008
234
showed increased FDG uptake in peripheral region of the mass.No other site of abnormal FDG uptake detected. The diagnosis of Malignant mesenchymal tumour with myogenic differentiation;Pleomorphic rhabdomyosarcoma was confirmed through histopathological study.of cystoscopic biopsy including immunohistochemistry (Fig.1). The rarity of case and need for newer drug were discussed with family. In view of advanced unresectable disease he was being treated with systemic chemotherapy along with target therapy. He received 3 cycles of chemotherapy (cyclophosphamide, liposomal adriamycin, VCR) on a 4 weekly schedule,given a loading dose of cetuximab (375 mg/m2) and then weekly 200 mg per sqm from May 08 to Aug 08. His repeat MRI and done in, July 08 revealed localized disease with clinical benefit in terms of effective pain (score 2/5) and bleeding control.However in view of continued unresectable status patient started on synchronous chemo-target therapy. DISCUSSION Prostate sarcoma is rare with only 183 cases reported in the English language medical literature from 1938-1998 [1-3]. Prostate sarcomas reach a much larger size before causing symptoms whereas sarcomas of the urinary bladder cause symptoms at smaller size and earlier stage [1]. Most patients with sarcoma of the prostate have symptoms related to bladder outlet obstruction. In 103 informative cases, 89 % of patients reported symptoms related to urinary obstruction. In 18% of cases, symptoms related to rectal
Case Report
S-2767/08
PASD
Vimentin
CK
PSA
235
Apollo Medicine, Vol. 5, No. 3, September 2008
Case Report
Desmin
MyoD1
PTAH
obstruction were observed. Major symptoms and characteristics of the prostate on palpation in prostate sarcoma in these 103 patients include 91 (89%) related with bladder outlet obstruction; 19 (18%) related to rectal obstruction; 6 (6%) pain; 4 (4%) hematuria; 1 (1) weight loss.
Fig. 1.
Morphology and immunohistochemistry for Pleomorphic Rhabdomyosarcoma of the prostate.
Apollo Medicine, Vol. 5, No. 3, September 2008
Characterization palpation (33 patients) include 21 (65%) smooth surface; 2 (6%) globular; 10 (29%) Irregular. In 40 patients 8 (21%) had soft or boggy consistency; 6 (16%) elastic of tense; 26 (63%) firm or hard [4]. This patient presented with features related to urinary and bowel obstruction and underwent suprapubic cystectomy. Diagnosis in usually made by needle biopsy and using immunohistochemistry as in this case. Prostate sarcoma may show an irregular hypoechoic prostate man with an anechoic area by ultrasonography [5-7]. A study involving these patients with RMS revealed that local extent of the disease could be delineated at the time of trans rectal needle
236
Case Report
biopsy [6]. Generally most prostate sarcoma are locally extensive but not metabolic at the time of diagnosis among 20 patients, 4 (20 %) showed metastatic disease at the time of diagnosis. The organ sites of metastases. The organ sites of metastases were long ( 2 cases [7,8] )], bone (2 cases [8,9]), and liver (1 case [3] ). In the absence of evidence of metastatic disease, aggressive local surgical excision should be performed. Most prostate sarcomas are usually quite large at the time of diagnosis. For locally extensive sarcomas involving the rectum, a total pelvic exenteration is indicated. Bilateral pelvic lymphadenectomy along with cysto prostatectomy is usually performed for respectable disease [1]. However this patient was locally advanced and unresectable throughout.
with adult prostatic sarcoma in world literature; also Pet Scan findings in p RMS and clinical benefit by chemo-target therapy has been likely described for first time in English literature. REFERENCE 1. Nicholas J Vogelzang, Peter T Scardino, William U Shipley, Frans MJ Debruyne, W Marsten Linehan. Comprehensive text book of genito urinary oncology. Chapter-55 (third edition ) 2005 Lippincott Williams & Wilkins. 2. Froehner M, Lossnitzer A, Manseck A, Koch R, Noack B, Wirth MP. Favorable long-term outcome in adult genitourinary low-grade sarcoma. Urology. 2000; 56(3):373-377. 3. Baker ME, Silverman PM, Korobkin M.Computed tomography of prostatic and bladder rhabdomyosarcomas. J Comput Assist Tomogr. 1985; 9(4): 780783.
Patient with low grade leiomyosarcomas with positive resection margins should receive post operative radiation therapy to lower the risk of local recurrence. Patients with high grade leiomyosarcomas or rhabdosarcomas and those with nodal metastases should generally receive adjuvant chemotherapy. A combination chemotherapy require that includes doxorubicin, seems most advisable, despite no clear evidence of benefit [1]. In the report of one of the largest single institution experiences, among six case treated with neoadjuvant chemotherapy or chemo radio therapy, three achieved size reduction of the primary tumor [10]. Leiomyosarcomas of the prostate (p LMS) have a better prognosis then prostatic rhabdomyosarcoma (p RMS) , with a 5 years survival of 42% for 53 cases of p LMS in contrast to a 13% 5 year survival rate for 21 cases of p RMS. No obvious improvement in survival rates for patients with PS was identified through more than 50 years [11]. Two reports of the largest single institution experience have revealed the existence of occasional long term survivors [10]. Negative surgical margin and absence of metastatic disease at presentation have been reported as predictive of long term survival [10]. The 5 years survival for prostate sarcomas was 28% out of total 97 patients, all of 21 patients with pRMS prostate died within 8 years. While a few survived out of 53 pLMS-P after 10 years [1]. This patient is undergoing treatment and intention is effective palliation. CONCLUSION This may be the first reported case of persistent thrombocytosis ,as an paraneoplastic syndrome associated
4. Dekernion JB, Paulson DF eds. Genitourinary cancer management, Herr HW. Sarcoma of the urinary tract. Philadelphia, PA: Lea and Fibger, 1987; 259-270. 5. Terris MK Transrectal ultrasound appearance of radiation-induced prostatic sarcoma Prostate.1998; 37:182-186. 6. Terris MK, Eigner EB, Briggs EM, Reese JH, Torti FM, Freiha FS. Transrectal ultrasound in the evaluation of rhabdomyosarcoma involving the prostate.Br J Urol. 1994; 74(3):341-344. 7. Yao JC, Wang WC, Tseng HH, Hwang WS.Primary rhabdomyosarcoma of the prostate. Diagnosis by needle biopsy and immunocytochemistry. Acta Cytol. 1988; 32(4): 509-512. 8. Bos SD, Slaa ET. An adult man with a rhabdomyosarcoma of the prostate. A case report.Scand J Urol Nephrol. 1991; 25(4): 329-330. 9. Waring PM, Newland RC. Prostatic embryonal rhabdomyosarcoma in adults: a clinicopathologic review Cancer 1992; 69: 755-762. 10. Sexton WJ, Lance RE, Reyes AO, Pisters PWT, Tu Shi-ming Pisters LL. Adult prostate sarcoma: The M.D. Anderson Cancer Center Experience. J Urology. 2001;166: 521-525. 11. Cheville JC, Dundore PA, Nascimento AG, Meneses M, Kleer E, Farrow GM, Bostwick DG. Leiomyosarcoma of the prostate. Report of 23 cases. Cancer. 1995; 76(8):1422-1427.
237
Apollo Medicine, Vol. 5, No. 3, September 2008