Not your typical male pelvic neoplasm

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1 Not your typical male pelvic neoplasm Poster No.: C-2589 Congress: ECR 2013 Type: Educational Exhibit Authors: V. Chidambaram, S. Philips, S. Thipphavong ; Toronto, ON/CA, San Antonio, TX/US Keywords: Pelvis, Genital / Reproductive system male, Ultrasound, CT, MR, Diagnostic procedure, Cancer, Neoplasia DOI: /ecr2013/C-2589 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 41

2 Learning objectives 1. To present an exhibit of atypical male pelvis neoplasms. 2. To present a series of well-documented cases of malignant lesions that can occur in the male pelvis including: chondrosarcoma, GIST, liposarcoma, rectal and prostatic leiomyosarcoma, adult rhabdoid tumour, and perianal alveolar rhabdomyosarcoma. 3. To present unusual cases of non-neoplastic male pelvic masses which can be confused with malignancy, including: inflammatory colorectal disease, seminal vesicle leiomyoma and seminal vesicle hemangioma. Background Occasionally, atypical male pelvic neoplasms can be encountered. These masses can be large at initial presentation and cross-sectional imaging such as MRI is required for further evaluation. Even with multimodality imaging work-up, these cases can be confusing or misdiagnosed if seen for the first time. Most large pelvic male masses are malignant. Atypical and rare pelvic benign neoplasms include: seminal vesicle hemangioma and leiomyoma. Ultimately, biopsy is usually required for definitive diagnosis. Imaging findings OR Procedure details MALIGNANT NEOPLASMS OF MALE PELVIS. This presentation does not include the typical prostatic malignancy, bladder transistional cell carcinoma or colorectal cancers. 1. Urachal Carcinoma. Adenocarcinoma represents less than 2 % of bladder neoplasms. They are sub classified as primary and secondary. Urachal adenocarcinomas form one third of the primary. 80 % of urachal cancers are adenocarcinomas. Rest are urothelial or squamous. In addition to hematuria and irritative symptoms, mucus may be secreted in urine in 25% of patients. It may also present with umblical discharge. Page 2 of 41

3 They are characteristically located at the bladder dome in the midline or slightly off midline. 90 % occur near bladder, rest along the course of urachus. Midline infraumblical soft tissue mass with calcification should be considered as an urachal adenocarcinoma until proven otherwise. On CT it is mixed solid and cystic in majority of cases. It is purely solid in about 16% of cases. Ct is sensitive for detecting calcification (72% of cases) which is commonly peripheral than stippled. Bulk of the tumor is extra vesical. Extravesical spread is common. Bladder wall invasion is seen in 92% of cases and metastasis in 48%. Rarely, pseudomyxoma peritonei may result from peritoneal carcinomatosis. On MRI the mucin is seen as focal areas of high T2W signal. The solid elements are isointense on T1W and enhance post contrast. For urachal adenocarcinoma an aggressive surgery to include cystectomy and en bloc resection of the urachal mass, posterior rectus fascia, peritoneum and abdominal wall is performed. Fig. 1: 1 References: - Toronto/CA 2. Ileal Conduit Recurrence. Page 3 of 41

4 Incidence of TCC recurrence locally is 3 to 16 % depending on disease stage. Stage 3b or those with lymph node involvement have higher risk. Local tumor recurrence has various patterns. They include pelvic soft tissue mass, obstructing ureteral stricture, pelvic lymphadenopathy and stricture at the urethra-intestinal anastomosis. Distant nodes including inguinal and aortocaval nodes should also be assessed on imaging. Fig. 2: 2 References: - Toronto/CA 3. Pelvic Scrotal Liposarcoma. Liposarcomas are bulky tumors similar to lipomas but generally more complex. Frequently they contain areas of fibrosis. On ultrasound they are usually echogenic but it is variable. On CT and MRI it is more specific with fat easily identified. It also helps in identifying both the fat and soft tissue components of the tumor on post contrast imaging. Benign lipomas and hernias containing omentum are mimics. Lipomas are generally Page 4 of 41

5 smaller. Hernias are elongated masses tracked back to inguinal canal. They are usually low grade well differentiated lesions spreading by local extension. High grade tumors can spread via lymphatics and hematogenously. In the scrotum they present as firm, slow growing, painless, fluctuant masses. Liposarcomas tend to be well circumscribed or encapsulated. They usually show a lobulated pattern. Small lobules become separated and become satellite nodules. Fig. 3: 3 References: - Toronto/CA 4. Rectal GIST. Gastrointestinal stromal tumors (GISTs) are rare. They constitute less than 3 % of GIT cancers. Less than 1% involve esophagus, colon and rectum. The rectal GISTs present with perineal mass or rectal bleeding due to ulceration. Tumor size and mitotic rates are used to assess benign versus malignant behaviour. Usually they are large at presentation. Page 5 of 41

6 Rectal GISTs usually heterogeneously enhance and are exophytic masses. They do not involve the bowel wall circumferentially. Therefore bowel obstruction is rare. They characteristically can have hemorrhage, necrosis or cyst formation. When at anorectal margin, local extension to ischiorectal fossa and prostate in men can occur. Low attenuation areas of hemorrhage and necrosis are present on CT. On T1W uniform intermediate signal, on T2W heterogeneous high signal with heterogeneous enhancement post contrast administration is seen. Differentials would include both epithelial and non-epithelial neoplasms of anorectal region. GISTs tend to have well defined margins and lack of perirectal adenopathy. Fig. 4: 4 References: - Toronto/CA Page 6 of 41

7 Fig. 5: 5 References: - Toronto/CA 5. Synchronous rectal and prostate cancers. Synchronous detection of prostate cancer and cancers of the lower third of rectum is uncommon. It is likely to increase with accurate pre-operative assessment and increased awareness for the potential for synchronous disease. Page 7 of 41

8 Fig. 6: 6 References: - Toronto/CA 6. Recurrent colon cancer invading prostate, seminal vesicles and having bone metastasis. Despite the widespread use of total mesorectal excision and perioperative radiotherapy, between 2.6% and 32% of patients with rectal cancer with will go on to develop a pelvic recurrence after initial curative surgery. There are yet to be any standardised surveillance programme post treatment. The highest risk is in the first 2 years. Most programs continue for further 3 years. Page 8 of 41

9 Fig. 7: 7 References: - Toronto/CA 7. Rectal tumor invading locally into the prostate. Locally advanced rectal cancers (T4 disease) involving the prostate and seminal vesicles are treated when possible with total pelvic exenteration. In some eligible patients, bladder sparing extended surgery can be performed. Page 9 of 41

10 Fig. 8: 8 References: - Toronto/CA 8. Mucinous rectal carcinoma. Histologically large extracellular mucin pools lined with malignant cells, cords and vessels giving a mesh like appearance. On MRI specifically the T2W fast SE the SI is higher than the non-mucinous tumors. On post contrast examination there may be heterogeneous enhancement of the mesh like structure. The mucinous tumors have a poorer prognosis, diagnosed at a higher grade, frequently recur locally and have higher incidence of local invasion. It should be distinguished from necrotic tumors, fluid collections or cysts. Page 10 of 41

11 Fig. 9: 9 References: - Toronto/CA 9. Leiomyosarcoma of rectum. Leiomyosarcoma of the anorectal region is extremely rare, comprising 5-7% of all leiomyosarcomas of the gastrointestinal tract. Leiomyosarcoma makes up only % of all malignancies of the colon and rectum. The tumour tends to spread either directly or via the bloodstream, but perirectal lymph nodes may be involved. Page 11 of 41

12 Fig. 10: 10 References: - Toronto/CA 10. Pelvic Chondrosarcoma. The pelvis is a common site for chondrosarcoma. Pelvic chondrosarcomas most frequently involve the ilium, with a particular in the region of the triradiate cartilage. Pelvic chondrosarcomas are often large lesions at initial evaluation owing to the delay in onset of clinical symptoms. CT or MR imaging invariably demonstrates aggressive features with cortical destruction and a large soft-tissue mass. Areas of matrix mineralization are frequently detected only on CT scans. Hip joint invasion and iliac adenopathy may also occur. Page 12 of 41

13 Fig. 11: 11 References: - Toronto/CA 11. Adult rhabdoid tumor of pelvis. Rhabdoid tumor is a rare highly aggressive tumor of kidney in pediatric population. Histologically it is of skeletal muscle origin. It is very rare in adults with no specific identifying features. Page 13 of 41

14 Fig. 12: 12 References: - Toronto/CA 12. Leiomyosarcoma of prostate. Sarcoma of the prostate is uncommon, representing only 0.1% of all malignant tumors of the gland. Leiomyosarcoma of the prostate is a rare primary malignant neoplasm of prostatic smooth muscle that typically follows an aggressive clinical course. It is the most common primary sarcoma of the prostate in adults, accounting for 38% to 52% of primary prostatic sarcomas. Page 14 of 41

15 Fig. 13: 13 References: - Toronto/CA 13. Perianal alveolar rhabdomyosarcoma. It is rare tumor occurring mainly in pediatric population and young adults. Rhabdomyosarcomas are of two types: embryonal and alveolar. Alveolar subtypes with metastatic disease have a very poor prognosis. MRI imaging is the technique of choice depicting tumor origin, size, local extent, invasiveness and nodal spread. Page 15 of 41

16 Fig. 14: 13 References: - Toronto/CA INFLAMMATORY AND BENIGN MALE PELVIC MASSES. There is potential for confusion with malignant processes and therfore affect management of patients. Usually tissue diagnosis is neccessary to differentiate. Obvious inflammtory causes like abscesses and diverticultis require imaging and clinical follow up. Resolution and stabilty are key to establish their benign nature in difficult cases. 1. Penile abscess. They can be life threatening. In addition to pain, there are systemic signs and raised inflammatory markers clinically. Treatment includes antibiotics, bilateral corporotomy, débridement, and placement of intracorporeal irrigation and suction drains. Acute purulent cavernositis often fails to respond to conservative therapy and requires penile amputation. In patients who recover, severe fibrotic cavernosal changes usually develop, producing irreversible erectile dysfunction. Page 16 of 41

17 Ultrasound plays a role in patients with inflammation to rule out abscess formation. Ct and MRI show extent of involvement of the perineum, abdominal wall and fascial planes. Abscesses appear hypoechoic on ultrasound. On MRI they are low on T1W and high on T2W with rim enhancement. On Ct they appear as fluid collection with peripheral enhancement. Fig. 15: 15 References: - Toronto/CA 2. Iliopsoas Schwannoma. Schwannomas are benign slow growing encapsulated tumors of nerve sheath origin. They account for 6% of all retroperitoneal tumors. Usually asymptomatic and large at presentation. On ultrasound, they are hypoechoic masses. Areas of cystic degeneration, calcification or hemorrhage may be seen on Ct or MRI. They are well defined with high SI on T2W, low on T1W. Contrast enhacement depends on cellularity. Page 17 of 41

18 Fig. 16: 16 References: - Toronto/CA 3. Seminal Vesicle leiomyoma Primary tumors of seminal vesicles are rare. Of the primary solid tumors, adenocarcinoma is the most common. A few cases of seminal vesicle leiomyoma have been reported in the literature. Page 18 of 41

19 Fig. 17: 17 References: - Toronto/CA 4. Seminal vesicle hemangioma. This was a well defined tumor diagnosed post biopsy. There are no reported cases in the literature to our knowledge. Page 19 of 41

20 Fig. 18: 18 References: - Toronto/CA 5. Inflammatory colorectal disease. It is important to distinguish inflammatory disease from malignant diseases. Diverticulitis can be difficult to evaluate for malignancy. Follow up is essential in these instances. Tissue biopsy when appropriate should also be performed to evaluate. Page 20 of 41

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41 Conclusion Ultrasound performed for vague pelvic pain in the male patient can result in the finding of an unusual and often large pelvic mass. Even with multimodality imaging and patient characteristics, making a definitive diagnosis can often be difficult. More often than not, large pelvic masses in males are likely to be malignant. Occasionally, these masses end up being an atypical benign neoplasm. However, biopsy and histology are typically required for definitive diagnosis. References 1. Uncommon Primary Pelvic Retroperitoneal Masses in Adults: A Pattern-based Imaging Approach. Shanbhogue et l. May 2012 RadioGraphics, 32, Imaging of the Seminal Vesicle and Vas Deferens Kim et al. July 2009 RadioGraphics, 29, Rhabdomyosarcoma in Pediatric Patients. McCarville et al. AJR June 2001 vol. 176 no Leiomyoma of the seminal vesicle. Shiotani et al. Jpn J Radiol (2009) 27: MR IMAGING IN THE DIAGNOSIS OF A RETROPERITONEAL SCHWANNOMA. Computerized Medical Imaging and Graphics, Vol. 18, No. 3, pp , I Leiomyosarcoma of the rectum: What role does adjuvant therapy play?john D Kiffer et al. Australasian Radiology(1999) 43, Treatment Strategies for Perianal Rhabdomyosarcoma: Report of Two Cases. Watanabe et al. Surg Today (2004) 34: DOI /s Personal Information Page 41 of 41