Nigerian Journal of Plastic Surgery

CASE REPORT
Year
: 2018  |  Volume : 14  |  Issue : 1  |  Page : 12--14

Diffuse hemangioma of penis, scrotum, perineum, and anal canal—A rare case report


Aditya P Singh1, Arun K Gupta1, Rajlaxmi Pardeshi2, Maryem Ansari3, Dinesh K Barolia1,  
1 Department of Pediatric Surgery, SMS Medical College Jaipur, Jaipur, Rajasthan, India
2 Department Obstetrics and Gynaecology, SMS Medical College Jaipur, Jaipur, Rajasthan, India
3 Department of Pathology, SMS Medical College Jaipur, Jaipur, Rajasthan, India

Correspondence Address:
Aditya P Singh
Near The Mali Hostel, Main Bali Road, Falna, Pali, Rajasthan
India

Abstract

Hemangiomas are benign tumors of the vascular endothelium and mucous membranes. Despite their common occurrence, especially during the infantile period, hemangiomas located in the genital region represent one of the rarest sites. Genital hemangiomas are frequently congenital but may emerge later in life. 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. We are presenting here a case of extensive penile shaft hemangioma in an 8-year-old male child with review of literature.



How to cite this article:
Singh AP, Gupta AK, Pardeshi R, Ansari M, Barolia DK. Diffuse hemangioma of penis, scrotum, perineum, and anal canal—A rare case report.Nigerian J Plast Surg 2018;14:12-14


How to cite this URL:
Singh AP, Gupta AK, Pardeshi R, Ansari M, Barolia DK. Diffuse hemangioma of penis, scrotum, perineum, and anal canal—A rare case report. Nigerian J Plast Surg [serial online] 2018 [cited 2024 Mar 29 ];14:12-14
Available from: https://www.njps.org/text.asp?2018/14/1/12/238818


Full Text

 INTRODUCTION



Hemangiomas are benign vascular malformations of enlarged dysplastic vascular channels with abnormal growth of the endothelial cells. Genital hemangiomas are divided into three major categories: capillary (superficial), histiocytoid (epithelioid), or cavernous. Capillary hemangiomas are generally benign lesions with distinct borders and no capsule, and show a web of capillary and immature vessel proliferation. The cavernous type is distinguished by the presence of large blood-filled spaces, whereas histiocytoid hemangiomas have atypical histiocyte-like endothelial cells and inflammatory infiltrate. 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] We are presenting here a rare case of diffuse hemangiomatosis of penoscrotal region, which extends into perineum, pelvis, and anal canal.

 CASE REPORT



An 8-year-old male presented to us with the complaints of discoloration of the scrotal wall, penis, and perineum with diffuse swelling of the right scrotum for last 3 years [Figure 1]. Scrotal swelling was compressible. There was no history of bloody stools or hematuria. The rest of the medical examination and patient history was unremarkable. Routine blood investigations were within normal limits. Ultrasonography (USG) color Doppler study showed diffuse echogenic nodular lesion in skin, subcutaneous, and muscle wall with minimal vascularity. No hemorrhage, cystic changes, calcifications were seen, and both the testes were normal. Magnetic resonance imaging (MRI) showed a heterogeneous soft tissue mass (90 × 69 × 53 mm) with multiple flow voids seen in shaft of penis extending in the wall of scrotum, root of scrotum, anterior perineum, right lateral wall of anal canal, anal verge, right perineum, right ischiorectal fossa, and right lateral wall of pelvis [Figure 2]. The mass was also involving root of corpora spongiosa, corpora cavernosa, and around proximal penile urethra. The patient was lost to follow-up. We discussed and offered the surgical treatment to the parent.{Figure 1}{Figure 2}

 DISCUSSION



The majority of hemangiomas present within the first 2 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.[2] Very few articles have been reported in literature describing penile hemangioma. A case of multiple 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 et al.[3]

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. Intrascrotal hemangioma has also been reported.[1]

Complications can be caused by consequences of the extension of the lesion to the rectum and bladder such as rectal bleeding and hematuria, ulceration with infection, hemorrhage due to trauma,[4] and the potential effect on the spermatogenic activity of the testicles. The studies conducted by Stahl et al.[4] and by Gotoh et al. demonstrated the harmful effect of the increased temperature of the hemangioma on the developing testicles.

Imaging studies must determine the vascularization of the lesion, its relation with intrascrotal structures, and its potential expansion to the penis, perineum, and structures of the lesser pelvis.[5]

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.[6]

In sonographic images, cavernous hemangiomas may appear hyperechoic or hypoechoic depending upon the content of the lesion (such as septa, blood containing units). Color Doppler may demonstrate blood flow within these lesions, but the absence of flow does not rule out the presence of these lesions. Computed tomography (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.[6] CT scans reveal a soft tissue mass with phleboliths. 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.[6] Presence of hematuria could indicate an expansion to the bladder wall.[7] In our case, such a symptom was not noticed.

Oral propranolol is considered as the first-line treatment for infantile hemangiomas.[8] If propranolol is given after the period of angiogenesis, it is not beneficial for the patient. On the other hand, when treatment starts at the stage of proliferation, the growth of the lesion is inhibited. Thus, it is possible that when propranolol is ineffective, the proliferation stage has passed. Early diagnosis and treatment, ideally within the first 6 months of life, are crucial.[8]

However, in cases of scrotal hemangiomas, many authors recommend the scheduled surgical removal of the lesion, with preservation of anatomic structures of the scrotum.[1],[5],[7] Enucleating the lesion with minimal removal of testicular tissue is recommended only in the case of an endoscrotal hemangioma.[9] In complicated superficial scrotal hemangiomas, a more conservative approach such as flash dye pulsed laser (CO2, Nd:YAG, yellow-light), infusion of NaCl 15%, and cryotherapy can be considered.[10] Flash dye pulsed laser is a promising alternative to surgery that can achieve a selective photothermolysis and destruction of the superficial vessels of the lesion.

 CONCLUSION



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.

ACKNOWLEDGMENT

The authors acknowledge Dr. Annu Bhandari, Radiologist, Senior Professor, SMS Medical College Jaipur for her support.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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3Nouira Y, Kbaier I, Attyaoui F, Menif E, Horchani A. Megapenis associated to corpus spongiosum agenesis with scrotal and pelvic hemangiomas. Eur Urol 2001;40:571-4.
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