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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 19  |  Issue : 4  |  Page : 614-618
Diffuse Cavernous Hemangioma of the Penis, Scrotum, Perineum, and Rectum - A rare tumor


Yash Diagnostic Center, Yash Hospital and Research Center, Moradabad, India

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   Abstract 

Hemangiomas are benign lesions that occur in any part of the body. Genital hemangioma involving the entire penis and scrotum are extremely rare. More rarely they can extend in to the pelvis making preoperative imaging imperative and decisive in treatment. Very few cases have been reported in the medical literature. Hereby, a rare cavernous hemangioma that involves the entire penis, scrotum and extends into perineum and rectum in an 18-year-old male is presented with review of literature.

Keywords: Cavernous, Hemangioma, Genital, Pelvis, Perineum, Rectum, Penis, Scrotum

How to cite this article:
Rastogi R. Diffuse Cavernous Hemangioma of the Penis, Scrotum, Perineum, and Rectum - A rare tumor. Saudi J Kidney Dis Transpl 2008;19:614-8

How to cite this URL:
Rastogi R. Diffuse Cavernous Hemangioma of the Penis, Scrotum, Perineum, and Rectum - A rare tumor. Saudi J Kidney Dis Transpl [serial online] 2008 [cited 2019 Nov 13];19:614-8. Available from: http://www.sjkdt.org/text.asp?2008/19/4/614/41323

   Introduction Top


Hemangiomas are benign vascular malfor­mations of enlarged dysplastic vascular chan­nels with abnormal growth of the endothe­lial cells. They are classified into capillary, cavernous, arteriovenous, venous and mixed sub-types. Cavernous and mixed varieties are the most common. Hemangiomas are the most common tumor of childhood, and they are most common in the musculoskeletal system, liver and spleen. Scrotal and penile (genital) hemangiomas are unusual anomalies, and comprise less than 1% of all heman­giomas and may extend into adjacent areas of the perineum, thigh, or anterior abdominal wall. [1]

The hemangioma may be congenital or secondary to neoplasia and trauma. Klippel­Trenaunay syndrome (KTS) is a congenital vascular anomaly characterized by limb hy­pertrophy, cutaneous hemangiomas, and vari­cosities.

The majority of hemangiomas present with­in the first two decades of life, and they may enlarge during the growth of the child. Clinically, they may manifest as either a faint blue patch or a soft, bluish vascular mass. It is usually mandatory to image the lesions and their extensions before surgical resec­tion, as physical examination is frequently inadequate to detect the extensions of the deep lesions. Ultrasound (US), computerized tomography (CT) scan, and magnetic reso­nance imaging (MRI) are recommended for the work-up.

Hereby, a rare case of diffuse cavernous hemangiomatosis of penoscrotal region, which extends into perineum and rectum, is pre­sented in the following case report.


   Case Report Top


An 18-year-old male presented with a 2­year history of diffuse, enlarging, soft tissue tumor with prominent superficial vessels in­volving the entire penis and perineoscrotal region for further evaluation. The chief com­plaint was of multiple soft tissue nodules deforming and enlarging the penis, and asso­ciated with poor erection and oligospermia. The nodules were compressible clinically, and vascular malformation was suspected. Pre­vious history of few episodes of bloody stools was present but there was no history of trauma or hematuria. The rest of the medical examination and patient history was unremarkable.

Previous US of the abdomen performed to evaluate bloody stools has revealed thicke­ning of the rectosigmoidal wall. Previous report of the US examination of the peno­scrotal region revealed multiple calcified foci along with multiple dilated tortuous channels limited to the skin. Blood flow was demon­strated on color Doppler imaging in some of these channels. However, the testes and penis were normal on US.

CT scan of the lower abdomen was per­formed including the penoscrotal, and the noncontrast images revealed multiple calci­fied lesions in the wall of the rectosig­moidal, perineal and penoscrotal regions, [Figure 1],[Figure 2]. However, the contrast en­hanced images revealed intensely diffuse mass that involves the wall of the rectum up to the rectosigmoid junction with luminal narrowing, [Figure 3]. There was an obvious extension into the perineum with continuation of the mass into the shaft of the penis and the wall of the scrotum, [Figure 3],[Figure 4], and [Figure 5]. In addi­tion, multiple discrete dilated tortuous vascu­lar channels were noted within the observed mass.

Based on the above radiological features, diffuse cavernous hemangioma that involves the penoscrotal region, perineum and rectum with phleboliths was diagnosed. Eventually, the patient underwent a combination of laser ablation and surgical excision with unevent­ful postoperative period. The biopsy of the excised specimen confirmed the lesion to be cavernous hemangioma.


   Discussion Top


Only a few number of articles describing penile hemangioma have been reported. Giant penile cavernous hemangioma with intrapelvic extension has been reported by Froehner M et al. [2] A case of mutiple hemangiomas of the scrotum, perineum and pelvis along with mega penis and associated with agenesis of the corpus spongiosum along with scrotal and pelvic hemangiomas have been reported by Nouira Y et al. [3] The findings observed in the cases described by the above authors are similar to that seen in the presented case.

Cavernous hemangioma of the scrotum primarily presents during childhood. Subcu­taneous scrotal-perineal hemangioma may mimic an inguinal hernia, thus forming a diagnostic and therapeutic challenge. [4] Large scrotal hemangioma may be associated with testicular damage believed to be due to the heat generated by hemangioma. [5] Intrascrotal hemangioma has also been reported. [1]

Diffuse cavernous hemangioma of the rec­tosigmoid is an uncommon disease that affects mainly young adults. As the main symptom is non-specific chronic rectal blee­ding, the clinical diagnosis is difficult and often delayed. [6] Other symptoms stem from a possible compression or invasion of adja­cent structures, such as lumbar or perianal pain, metrorrhagia, hematuria, etc. Larger lesions may be associated with coagulation disturbances. A large number of these mal­formations are missed or falsely diagnosed, since symptoms are attributed to internal hemorrhoids, polyps, ulcerative colitis, etc. [7]

Imaging can help assessing the extent of the hemangioma, as well as detecting any associated abnormalities. The most typical radiographic finding is a soft-tissue mass or prominence containing phleboliths (small cal­cifications). Presence of phleboliths is highly suggestive of cavernous hemangioma. [8] Phle­boliths are usually better demonstrated on plain radiographs or CT scans.

In sonographic images cavernous heman­giomas may appear hyperechoic or hypo­echoic depending upon the content of the lesion (such as septa, blood containing units). Phleboliths may be seen as echogenic foci with distal acoustic shadowing. Color Doppler may demonstrate blood flow within these lesion but the absence of flow does not rule out the presence of these lesions.

CT and MRI provide a simple, noninva­sive method of diagnosing and determining the extent of these lesions in addition to delineating their relationship with adjacent structures. Therefore, these are the imaging techniques of choice for this condition and are considered mandatory before surgical procedures are taken. [7] CT scans reveal a soft tissue mass with phleboliths. In the rectum and sigmoid, they are seen as no­dular thickening of the wall with phle­boliths. Involvement of the urinary bladder may cause hematuria. A non-homogeneous, subtle to intense enhancement of the lesion is evident on postcontrast images. [9]

On MRI, the lesions are disclosed as soft tissue masses with lobulated margins that is hypointense on T1-weighted images and hy­perintense on T2-weighted images. In addi­tion, they have multiple, rounded, signal voids that represent phleboliths. Vascular signal voids and flow-related enhancement are un­common features. Fluid-fluid levels may rare­ly be present, although it is more suggestive of lymphatic malformation. Penile MRI is more useful to demonstrate the anatomy of the penis and to delineate the extent of these lesions in the penile shaft.

Selective arteriography may detect the lesions, but it is of little importance for diag­nosis. In addition, it reveals normal results in most patients, because of the presence of thrombosis in dilated vascular spaces within the hemangioma. Recently, it has been sug­gested that radionuclide studies, particularly Tc-99 scans, may play a role in the assess­ment of the extension of these lesions. [7]

Once a diagnosis has been established, era­dication of the lesion should be recommen­ded. Treatment options include a sclerosing agent (such as alcohol), surgery or laser therapy. In one study, the success rate of laser treatment was 92.8% with a low immediate complication rate (approximately 3.57%) that included minimal scarring and deformity. No long-term complications of laser therapy were noted. The study con­cluded that laser treatment of these lesions enables good results with a very low inci­dence of complications. Surgery and other treatment modalities are not always satis­factory, yield similar or less efficient res­ults, and have a higher complication rate. Laser treatment may be the treatment of choice in some settings. [10] For rectosigmoid hemangioma, commonly used surgical techniques include abdominal-perineal resection of the lesion and "sleeve" coloanal anasto­mosis, [7] which offers major advantages such as a lower risk of intraoperative bleeding, no risk of damaging the pelvic nerves, spa­ring of continence, and avoidance of a per­manent colostomy. It should therefore be considered the treatment of choice for this uncommon condition. [11]

In conclusion, cavernous hemangioma that involves penis and scrotum is rare and may be associated with extension into the peri­neum, rectum and sigmoid colon, which may present diagnostic and treatment difficul­ties. Imaging studies can determine the ex­tent of the lesion, delineate its relationship with adjacent structures, and help planning therapy and follow up.

 
   References Top

1.Lin Y, Sun GH, Yu DS, et al. Intrascrotal hemangioma. Arch Androl 2002;48(4):259-65.  Back to cited text no. 1    
2.Froehner M, Tsatalpas P. Giant penile cavernous hemangioma with intrapelvic extension-review of the literature. Urology 1999;53(2):414-5.  Back to cited text no. 2    
3.Nouira Y, Kbaier I, Attyaoui F, Menif E, Horchani A. Megapenis associated to corpus spongiosum agenesis with Scrotal and Pelvic Hemangiomas. Eur Urol 2001;40(5): 571-4.  Back to cited text no. 3    
4.Ferrer FA, McKenna PH. Cavernous hemangioma of the scrotum: a rare benign genital tumor of childhood. J Urol 1995;153 (4):1262-4.  Back to cited text no. 4    
5.Gotoh M, Tsai S, Sugiyama T, Miyake K, Mitsuya H. Giant scrotal hemangioma with azospermia. Urology 1983;22(6):637-9.  Back to cited text no. 5    
6.Djouhri H, Arrive L, Bouras T, et al. Diffuse cavernous hemangioma of the rectosigmoid colon: imaging findings. J Comput Assist Tomography 1998;22(6):851-5.  Back to cited text no. 6    
7.Hervias D, Turrion JP, Herrera M, et al. Diffuse cavernous hemangioma of the rectum: an atypical cause of rectal bleeding. Rev Esp Enferm Dig 2004;96(5):346-52.  Back to cited text no. 7    
8.Yeoman LJ, Shaw D. Computerized tomo­graphy appearances of pelvic hemangioma involving the large bowel in childhood. Pediatr Radiol 1989;19(6-7):414-6.  Back to cited text no. 8    
9.Perez C, Andreu J, Llauger J, Valls J. Hemangioma of the rectum: CT appearance. Abdom Imaging 1987;12(1):347-9.  Back to cited text no. 9    
10.Sarig O, Kimel S, Orenstein A. Laser treatment of venous malformations. Ann Plast Surg 2006;57(1):20-4.  Back to cited text no. 10    
11.Londono-Schimmer EE, Ritchie JK, Hawley PR. Coloanal sleeve anastomosis in the treatment of diffuse cavernous hemangioma of the rectum: long-term results. Br J Surg 1994;81(8):1235-7.  Back to cited text no. 11    

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Correspondence Address:
Rajul Rastogi
C-002, Upkari Apartments, Plot no 9, Sector 12, Dwarka, Delhi, 110078
India
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PMID: 18580022

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures
 

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