Congenital hemangioma (RICH)

 
Congenital hemangioma or Rapidly Involuting Congenital Hemangioma (RICH) is a similar benign tumor (birthmark) to infantile hemangioma, but it differs from infantile hemangioma in that it has a fully developed presentation at birth, with rapid involution and regression. Overall, congenital hemangioma is a rare entity.

                        

Photo#1 shows a moderately sized congenital hemangioma (birthmark) or rapidly involuting congenital hemangioma in the thigh. There are central ulcer like skin changes. Photo#2 shows a similar congenital hemangioma lesion in the scalp. 

These birthmarks may be difficult to differentiate from other congenital lesions, particularly from congenital fibrosarcoma. Recognizing these congenital birthmarks and differentiating them from other congenital lesions or tumors is very important to reduce unnecessary invasive diagnostic procedures (surgical biopsies or major surgical excision procedures) in these newborns, because most of these birthmarks involute rapidly. 

 

Diagnostic Tests?  The appropriate diagnostic test is ultrasonography with Doppler and/or magnetic resonance imaging (MRI). The imaging features of these tumors (birthmarks) also greatly differ from more common types of infantile hemangiomas, but may be confused with arteriovenous malformations (AVM) or other tumors (fibrosarcoma etc.). Various sized cystic vascular lesions with arteriovenous connections similar to high-flow lesions (AVM) are commonly seen on ultrasound examinations or MRI scans. Interpretation of these scans (US or MRI) by an experienced radiologist or interventional radiologist is important for accurate diagnosis. Some tumors may require tissue sampling (biopsy) for definite diagnosis.

 

Figure#1 (T1 weighted MRI image) shows a large lesion (rapidly involuting congenital hemangioma) in the left upper thigh with multiple dark vessels due to rapid blood flow, unusual appearance for common infantile hemangiomas. Figure#2 is an ultrasound image of rapidly involuting congenital hemangioma. There are multiple high-flow relatively large vessels in the tumor tissue.  Figure#3 is an catheter arteriogram of rapidly involuting congenital hemangioma (in the head). There are multiple aneursyms and arteriovenous connections similar to AVM. 

What is the treatment ?

These lesions (birthmarks) usually rapidly involute (shrink), usually much faster than common hemangiomas. However, some patients may develop significant health problems (e.g., cardiac failure, bleeding similar to kaposiform hemangioendothelioma) due to arteriovenous shunting. Therefore, patients with these rare birthmarks need to be followed closely and appropriate therapeutic intervention should be implemented whenever needed. Embolization should be used if needed. Medical intervention (pharmacological) is similar to that of infantile hemangiomas.

Photo#1 is at 6 days, photo#2 at 12 days, photo#3 at 45 days, and photo#4 at 8 months. This patient was treated with systemic steroids for a few months. 

 

 
 
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Recent Literature

  • Case Studies concluded that RICH is difficult to distinguish angiographically from arteriovenous malformation and congenital fibrosarcoma >>> more
  • Clinical and histopathologic features of RICH were evaluated and concluded that RICH, NICH (non-involuting congenital hemangioma), and common infantile hemangioma have overlapping clinical and pathologic features. These observations support the hypothesis that these vascular tumors may be variations of a single entity ab initio. It is unknown whether the progenitor cell for these uncommon congenital vascular tumors is the same as for common infantile hemangioma >>> abstract
  • Study evaluating 10 patients with lesions consistent with the clinical entity of rapidly involuting congenital hemangioma (RICH) concluded that RICH can be reliably diagnosed in most cases with a consideration of the unique clinical features and with the use of noninvasive imaging studies >>> abstract
 
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