ABSTRACT

CORRESPONDING AUTHOR:
Dr. M. Ramani,
Professor, Department of Pathology,
Niloufer Hospital, Institute Of Child Health,
Andhra Pradesh, Hyderabad, India.
Email- drmramani@sify.co

ABSTRACT: BACKGROUND: Traditionally there has been much debate about vascular lesions being developmental malformations or types of neoplasia. Vascular  tumours  are  believed  to  result  from developmental  errors  that  occur  at  4-10  weeks of gestation.  Most cases are sporadic. Vascular  malformations  result  from  abnormal sized  or  abnormal  number  of  vascular  structures.  These  malformations  usually  manifest  as cutaneous  birthmarks  and  have  had  a  number  of  classifications,  producing  a  range of  confusing  terms. AIMS AND OBJECTIVES: To study the incidence, classification and spectrum of various vascular lesions in children with a clinicopathological perspective. MATERIAL AND METHODS: The present retrospective study was undertaken at the Department of Pathology, Paediatric Referral Hospital, during the period from June 2009 to May 2011. About 44 patients of the paediatric age group were analysed for clinical details and pathological diagnosis. Vascular lesions were classified histopathologically according to International Society for the Study of Vascular Anomalies (ISSVA) classification broadly into two groups, mainly vascular tumours and malformations. OBSERVATION AND RESULTS: Of the 44 cases reported in our study, vascular lesions were commonly seen in infants. The male to female ratio was 1:2.1.  Most common site involved was head and neck region (73%). 39 cases were classified as vascular tumours which included haemangiomas and 5 classified as vascular malformations which included lymphangiomas. CONCLUSION: Vascular  lesions  though  clinically  diagnosed  only  as  haemangiomas, represent  pathologically  varied  entities.  These  lesions  when  present  in  infants  and  in functional  areas,  their  precise  diagnosis  by  the  pathologists  makes  a  significant difference  to  the  management  of  these  simple  looking  vascular  lesions  of  the  children.

KEYWORDS: International Society for the Study of Vascular Anomalies (ISSVA) classification, Haemangiomas, Lymphangiomas.

m

INTRODUCTION: Vascular lesions are abnormalities of vascular elements that presents at birth or during infancy. The estimated prevalence of these vascular anomalies is 4.5% ^[1]. Traditionally there has been much debate about vascular lesions being either developmental malformations or types of neoplasia ^[2].

Vascular  tumours  are  said  to  result  from developmental  errors  that  occur  at  4-10  weeks gestation.  Most of the cases are sporadic and are sometimes occasionally inherited in an autosomal dominant fashion. Vascular  malformations  result  from  abnormal sized  or  abnormal  number  of  vascular  structures.  They  usually  manifest  as cutaneous  birthmarks  and  have  had  a  number  of  classifications,  producing  an  array of  confusing  terms ^[3].

Mulliken and Glowacki (1982) suggested a dualistic classification for vascular anomalies based on pathological features. International Society for the Study of Vascular Anomalies (ISSVA) adopted this classification in 1996 and expanded it. Since then this classification is widely accepted as it shows a systematic approach towards vascular lesions that correlates predictably with clinical history, course of the disease and treatment decisions.

For the diagnosis of vascular lesions in children, medical history and physical examination are important. Emphasis has to be laid on precise histopathological report, meticulous clinical and radiological assessment ^[5]. Histopathology helps in confirming  the  clinical  diagnosis, while imaging aids in diagnosis, estimating  the  extent  of  the  lesion,  and  determining  the  feasibility  of  surgical resection.

The present study reviews the incidence, classification and spectrum of various vascular lesions in children with a clinicopathological perspective.

MATERIAL AND METHODS: The  present  study  of  vascular  lesions  in  children is  a  2  year  retrospective  study  done  at  Department of  Pathology,  Paediatric  Referral  Hospital from June  2009  to  May  2011.  The  study  group consisted  of  44  patients  of  the  paediatric  age  group,  14  of  them  being  boys  and 30  girls.  They were analysed for clinical details and pathological diagnosis.  The  materials  for  the  study  comprised  of specimens  received  at the  Department  of  Pathology. Biopsies of surgical specimens were formalin fixed, paraffin embedded and sectioned. The slides were stained with Haematoxylin and Eosin and were examined under light microscopy. Vascular lesions were histopathologically classified according to ISSVA classification into two major categories which included vascular tumours and malformations.

RESULTS: In the present study, we reviewed 44 cases of vascular lesions occurring in children. Vascular lesions were commonly encountered in infants. There were 30 girls and 14 boys. Sex incidence clearly showed female preponderance with Male: Female ratio of 1: 2.1. Table 1 and 2 shows age and sex incidence of all the vascular lesions.

Commonest site involved by vascular lesions was head and neck (32/44, 73%), followed by upper extremity (4/44, 9%) and trunk (4/44, 9%). The other sites were lower extremity (3/44, 7%) and anogenital region (1/44, 2%). (Table 3)

Histopathological assessment was done according to ISSVA classification. Vascular lesions in our study were broadly classified into vascular tumours and malformations. Increased endothelial cell turnover was witnessed in vascular tumours and was not appreciated in vascular malformations. Increased endothelial cell turnover was assessed histopathologically under light microscopy by documenting increased mitoses. Of the 44 cases we reported, 39 cases were classified as vascular tumours which included haemangiomas (Figure 3A, 4A, 4B, 3B) and 5 cases were classified as vascular malformations which included lymphangiomas (Figure 5A, 5B). Figure 1 Shows bar diagram with vascular lesions.

In the present study, vascular tumours which included haemangiomas occurred predominantly in girls. 28 of 44 girls presented with haemangiomas. (Figure 2)

Complications were seen in 4 cases (2.95%). 2 cases  presented  with  ulcer,  1 with  abscess  and  1  with  bloody  discharge.  The  site  of  the  lesion  was  scalp  in  2 cases,  arm  in  1  and  chest  in  1  case.  All 4 patients were girls with haemangiomas.

DISCUSSION: Vascular lesions have been designated a variety of names since nineteenth century. They were thought to be formed due to longing of the mother for particular things or her dislike to them, hence these marks resembled mulberries, strawberries, grapes, pines or bacon. Virchow regarded them as vascular tumours and classified them based on channel architecture into angioma simplex, cavernosum and racemosum ^[1].

ISSVA classification system divides vascular anomalies into two categories namely vascular tumours and malformations. The major difference between two categories is based on increased endothelial cell turnover. Vascular tumours have increased endothelial cell turnover while vascular malformations do not. Vascular malformations are structural abnormalities of the capillary, venous, lymphatic and arterial system that grow in proportion to the growth of child ^[4].

In the present study, vascular lesions were commonly encountered in infancy. Sex incidence clearly showed female preponderance with Male: Female ratio of 1: 2.1. These findings were consistent with other studies ^{[6, 7, 8, 9, 10]}.

The most common site involved by the vascular lesions in our series was head and neck (73%) followed by trunk (9%), upper extremity (9%), lower extremity (7%) and anogenital region (1/44, 2%). In one series about 60% of vascular lesions occurred on the head and the neck, 25% on the trunk, and 15% on the extremities ^[11]. Studies by other authors showed similar findings ^{[9, 10]}. Our findings are consistent with the available literature.

Mulliken et al. (1982) reviewed 49 specimens from variety of vascular lesions for their cellular features. There were two major categories of lesions that emerged from this review which included vascular tumours like hemangiomas (26 cases) and vascular malformations (26 cases) ^[2].

In one large series, 1127 cases of vascular lesions were evaluated and classified into hemangiomas (969 cases) and malformations (158cases) ^[12].

Al-Adnani et al.^[13] (2006) evaluated 144 cases of paediatric vascular anomalies which included 70% classified as vascular tumours and 30% classified as vascular malformations according to International Society for the Study of Vascular Anomalies (ISSVA) classification.

According to another study, vascular lesions were classified into hemangiomas (84%) and vascular malformations (16%)^[10].

In this present study, of the 44 cases reported, 39 cases were classified as vascular tumours which included haemangiomas and 5 cases as vascular malformations which included lymphangiomas. Our results were consistent with available literature.

Haggstrom et al. (2006) reported complications in cases with haemangiomas. Ulceration was noted in 23.2 % of the cases, visual compromise in 6.9 %, airway obstruction in 1.8%, auditory canal obstruction in 1.1% and cardiac compromise was seen in 0.4% ^[14]. In our study, complications were seen in 4 cases of haemangiomas. Of these, 2  presented  with  ulcer,  1 with  abscess  and  1  with  bloody  discharge.

CONCLUSION: Vascular  lesions  although  clinically  diagnosed  only  as  haemangiomas, represent  pathologically  diverse  entities. Haemangiomas do not appear  as  simple  as  they  sound  and  present  with wide range of diagnostic  dilemmas. Morphologically they exhibit varied pictures. It is important for pathologist to differentiate them into vascular tumours and malformations. These  lesions  when  present  in  infants  and  in functional  areas,  their  precise  diagnosis  by  the  pathologists  makes  a  significant difference  to  the  management  of  these  simple  looking  vascular  lesions  of  the  children.

REFERENCES:

1. Greene AK. Vascular anomalies: current overview of the field. Clin Plast Surg. 2011 Jan; 38(1):1-5.
2. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982 Mar; 69(3):412-22.
3. Blei F, Walter J, Orlow S J, Marchuk DA. Familial segregation of hemangiomas and vascular malformations as an autosomal dominant trait. Arch Dermatol. 1998 Jun; 134(6):718-22. Erratum in: Arch Dermatol 1998 Nov; 134(11):1425.
4. Lowe LH, Marchant TC, Rivard DC, Scherbel AJ. Vascular malformations: classification and terminology the radiologist needs to know. Semin Roentgenol. 2012 Apr; 47(2):106-17.
5. Paula E. North. Pediatric Vascular Tumors and Malformations. Surgical Pathology Clinics; September 2010; Vol. 3, Issue 3, Pages 455-494.
6. Beth A. Drolet, Nancy B. Esterly, and Ilona J. Frieden, Hemangiomas in Children. N Engl J Med 1999; 341:173-181.
7. Chiller KG, Passaro D, Frieden IJ. Hemangiomas of infancy: clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex. Arch Dermatol. 2002 Dec; 138(12):1567-76.
8. Viswanathan V, Smith ER, Mulliken JB, Fishman SJ, Kozakewich HP, Burrows PE, Orbach DB. Infantile hemangiomas involving the neuraxis: clinical and imaging findings. AJNR Am J Neuroradiol. 2009 May; 30(5):1005-13.
9. Ye CS, Pan LX, Huang YB, Han AJ, Ye RY, Li SQ, Li XX, Lu WM, Wang SM. Clinical analysis of vascular anomalies: a hospital-based retrospective study of 592 patients in southeast China. Chin Med J (Engl). 2011 Oct; 124(19):3008-12.
10. Stanciulescu, Maria-Corina, Emanuela Verenca, E. S. Boia, C. M. Popoiu, V. L. David, Anca Popoiu, Patricia Cristodor. Vascular anomalies in children–17 years’ experience. Jurnalul Pediatrului, july-december 2012, Year XV, Vol. XV, Nr. 59-60:79-83.
11. Oak SN, Viswanath N. Management of hemangiomas in children. Indian J Dermatol Venereol Leprol 2006; 72:1-4.
12. Akyuz C, Yaris N, Kutluk MT, Buyukpamukcu M. Benign vascular tumors and vascular malformations in childhood: a retrospective analysis of 1127 cases. Turk J Pediatr. 1997 Oct-Dec; 39(4):435-45.
13. Al-Adnani M, Williams S, Rampling D, Ashworth M, Malone M, Sebire NJ. Histopathological reporting of paediatric cutaneous vascular anomalies in relation to proposed multidisciplinary classification system. J Clin Pathol. 2006 Dec; 59(12):1278-82.
14. Haggstrom AN, Drolet BA, Baselga E, Chamlin SL, Garzon MC, Horii KA, Lucky AW, Mancini AJ, Metry DW, Newell B, Nopper AJ, Frieden IJ. Prospective study of infantile hemangiomas: clinical characteristics predicting complications and treatment. Pediatrics.2006 Sep; 118(3):882-7.

Table 1: Age Distribution.

Table 2: Sex Distribution

Table 3: Distribution of Lesions at different locations.

Figure 1: Bar diagram showing the percentage of various lesions.

Figure 2: Bar diagram showing the sex incidence in different vascular lesions.

Figure 3: (A) Photomicrograph of Haemangioma (Haematoxylin and Eosin, 10X) (B) Photomicrograph ofHaemangioma (Haematoxylin and Eosin, 10X)