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| Year : 2008 | Volume
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| Issue : 4 | Page : 614-618 |
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| Diffuse Cavernous Hemangioma of the Penis, Scrotum, Perineum, and Rectum - A rare tumor |
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Rajul Rastogi
Yash Diagnostic Center, Yash Hospital and Research Center, Moradabad, India
Click here for correspondence address and email
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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 2022 Aug 10];19:614-8. Available from: https://www.sjkdt.org/text.asp?2008/19/4/614/41323 |
| Introduction | |  |
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 | | |
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 | | |
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 | | |
| 1. | Lin Y, Sun GH, Yu DS, et al. Intrascrotal hemangioma. Arch Androl 2002;48(4):259-65.  |
| 2. | Froehner M, Tsatalpas P. Giant penile cavernous hemangioma with intrapelvic extension-review of the literature. Urology 1999;53(2):414-5. |
| 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. |
| 4. | Ferrer FA, McKenna PH. Cavernous hemangioma of the scrotum: a rare benign genital tumor of childhood. J Urol 1995;153 (4):1262-4. |
| 5. | Gotoh M, Tsai S, Sugiyama T, Miyake K, Mitsuya H. Giant scrotal hemangioma with azospermia. Urology 1983;22(6):637-9. |
| 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. |
| 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. |
| 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. |
| 9. | Perez C, Andreu J, Llauger J, Valls J. Hemangioma of the rectum: CT appearance. Abdom Imaging 1987;12(1):347-9. |
| 10. | Sarig O, Kimel S, Orenstein A. Laser treatment of venous malformations. Ann Plast Surg 2006;57(1):20-4. |
| 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. |
Correspondence Address:
Rajul Rastogi
C-002, Upkari Apartments, Plot no 9, Sector 12, Dwarka, Delhi, 110078
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 18580022 
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5] |
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