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CASE REPORTS
BENIGN JUVENILE HAEMANGIOMA : CASE REPORT OF AN UNUSUAL PRESENTATION
KAILASH DEVALIA*, RUJUTA MEHTA**, MG YAGNIK***
INTRODUCTION
Haemangiomas are non-cancerous vascular tumours which commonly appear at birth or shortly after birth i.e.within the first month or neonatal period.Their rapid growth can be very alarming to parents. The commonest sites are the skin and subcutaneous tissues, followed by the deep fascia, occasionally are intra-muscular and are exceptionally rare in bone. Haemangioma is one of the most common soft tissue tumours comprising 7% of all benign tumours.2 Intramuscular haemangiomas are rare, making up 0.8% of all haemangiomas.The clinical picture in intra-muscular tumours can be varied, confusing and at times misleading particularly if it presents at an early age. This case is presented because of the rarity of the incidence of the intra-muscular haemangioma, the difficulty in establishing a definitive in diagnosis and yet surprisingly easy mode of treatment.
CASE REPORT
A 2 month old child presented with circumferential right thigh swelling and erythema (Fig. 1), discoloration, short duration fever and peno-scrotal oedema, with sudden and enormous increase in size of swelling, to the paediatric orthopaedic clinic at Bai Jerbai Wadia Children Hospital. The child was referred from Jalna with a high degree of suspicion about a rapidly progressing malignant tumour. The swelling was warm and indurated with moderate tenderness and good knee range of motion. Distal neurovascular status was normal. Haematological investigations were normal. Plain radiographs revealed a diffuse and slight increase in soft tissue shadow around the lower and middle third of femur with no bony involvement (Fig. 2), no periosteal reaction and hence were inconclusive. USG showed few hypo-echoic shadow with hypervascularity. MRI was inconclusive of diagnosis, showing circumferential mass in the quadriceps (lateral more than medial) and lateral hamstrings with homogeneous density (Fig. 3). The differential diagnosis therefore pointed towards either sub-acute osteomyelitis, Ewing's sarcoma, fibrosarcoma or any other tumorous condition. The child underwent a diagnostic biopsy.
During this procedure profuse bleeding occurred. Intra-operative findings revealed a fatty vascular tissue surrounding almost the entire girth of lower right thigh. The histo-pathology was reported as Benign Juvenile Intramuscular Haemangioma. Angiographic embolisation of the feeder by cyanocrylate glue was planned as a proposed treatment but child was clinically unfit at that time due to eosinophilia.
Follow-up
The child returned after 3 months with partial regression in the size of swelling. 80% regression by 6 mths. of age and complete regression at 1yr (Fig. 4). At subsequent follow-ups at 2 and 3 yrs the swelling has remained totally resolved with no evidence of recurrence (Fig. 5).
DISCUSSION
These benign vascular swellings are aetiologically hamartomatous malformations of normal vascular tissues and are considered by many authors to represent benign neoplasms. Females more often are affected than males.7 The type of tumour usually is classified by the type of vessels present within them. Capillary haemangiomas are composed of small mature vessels but when the vessels become massively dilated they are called cavernous haemangiomas. The presence of arteriolar or arterial structures allows the use of the term arteriovenous haemangiomas and the presence of dilated thrombosed vessels provides a diagnosis of papillary endothelial hyperplasia. They arise in childhood and adolescence.The superficial lesions arising from skin and subcutaneous tissues present
as painless masses with distinctive bluish tinge.They are soft and easily compressed. The deep lesions from fascia and muscles present, because of intermittent but persistent discomfort. They seldom have any physical signs. Intra-muscular haemangiomas are uncommon compared to other types of haemangiomas. Watson and McCarthy estimated that intramuscular haemangiomas accounted for 0.8% of all benign vascular tumours. Intra-muscular haemangiomas occur most often in young adults, with 80-90% presenting in individuals younger than 30 years. They occur most often in the lower extremities, especially the thigh, and typically present with a palpable mass, but the overlying skin typically is not discolored. Shortly after birth some grow rapidly, out of proportion to the growth of the child almost at a very alarming rate. It has also been well documented that they undergo a period of involution during which they spontaneously become smaller and lighter in colour. This gradual process of involution may take several years. Some haemangiomas disappear completely and others undergo only a partial involution.
Of the imaging modalities X-ray and CT scan are unrewarding. Characteristic calcifications with phleboliths occur most frequently in cavernous haemangiomas (30% of lesions).1,2 MRI defines the extent of the swelling and its relationship from adjacent muscles and other soft tissues. Haemangiomas show increased signal on both T1 and T2 weighted images, frequently with areas of signal void. These void areas may be indicative of dense fibrous tissue, thrombi, phleboliths, or regions of high flow. It is noteworthy that in our case the MRI completely failed to give any clue towards a concrete diagnosis. This can probably be explained by the fact that this swelling was possibly of the low-flow variety in which bleeding had already occurred within the tumour as witnessed by the sudden increase in size over a week prior to the child presenting to us: and thus no vascular channels were identifiable on the MRI scan.1,2 Angiograghy especially DSA, not only clinches the diagnosis readily but also accurately identifies the pattern and the extent of the haemangiomatous neovasculature. The lesions may be high flow or low flow. This distinction between high flow and low flow can be important in treatment decisions, as high-flow lesions are more likely to benefit from embolization than are low-flow lesions. However it is technically difficult to perform in children especially below 1 yr of age and therefore was deferred in our case till a definite diagnosis was established.
The most important complication especially in a high flow type of malformation is spontaneous profuse bleeding. Haemangiomas do not undergo malignant transformation. In children and adolescents, the majority of haemangiomas are benign. Occasionally they will permeate through all the tissue barriers in an aggressive fashion.Treatment
Superficial lesions are usually amenable to surgical excision. Intra-capsular excision is often followed by recurrence as the lesion rarely forms a pseudocapsule. In theory extra-capsular excision should provide a definitive procedure but it is impossible to dissect between the periphery of the lesion and the normal tissues without inadvertent transection of occult extensions. Wide excision does not always lead to complete cure, and is often injustified due to excessive morbidity. Recurrence rates following surgery range from 18-50%.
Injection with sclerosing agents and angiographic embolisation of the feeder are the new modalities of treatment which also includes cryosurgery. A combined method of embolisation and cryodestruction has been documented to treat and decrease the recurrence of difficult haemangiomas.4 Embolization may be used preoperatively to decrease intraoperative blood loss and postoperative recurrence. Newer modalities of treatment include graded injections of interferon which has recently shown a very high rate of rapid regression with the least amount of morbidity. Daily subcutaneous injections of interferon alfa-2a to a target dose of 3 million U/m2 of body surface area for a minimum of 4 months is an effective treatment though neurological evaluation has to be done.5,7 Laser knife excision of haemangiomas7 is a recently reported technique developed to better control intraoperative bleeding for these vascular processes.
Pre-operative ultrasoundguided hook-wire localization may aid in defining the extent of a haemangioma for intraoperative guidance during excision. Radiation has been used to treat soft tissue haemangiomas in surgically inaccessible or potentially dangerous sites.
CONCLUSION
We reiterate therefore that in any infant with a clinical picture mimicking an aggressive tumour the possibility of a haemangioma must be kept in mind. Often, intramuscular haemangiomas cannot be definitively distinguished from soft tissue sarcomas, especially hemangiopericytoma, based of clinical examination alone.6 Since it poses a difficulty in establishing an accurate diagnosis an MR-angiography scan must be a part of the investigative armamentarium. These lesions can be treated with a variety of lesser morbid options like interferon or therapeutic embolisation, and surgical excision should be reserved only for small superficial well demarcated lesions as the last option, since it is possible to have complete resolution of the vascular malformation by conservative techniques.
REFERENCES
1. Mark D Murphey, Julia K Fairbairn, Linda M Parman, et al. Musculoskeletal Angiomatous Lesions : Radiologic-Pathologic Correlation. Radiographics 1995; 15 : 4.
2. Allen PW, Enzinger FM. Hemangioma of skeletal muscle:an analysis of 89 cases. Cancer 1972; 29 : 8-22.
3. Cohen AJ, Youkey JR, Clagett P, et al. Intramuscular hemangioma. JAMA 1983; 249 : 2680-82.
4. Medical Shafranov VV, et al. Treatment of Hemangiomas In Children By Cryomethod Proceedings 10th World Congress
of Cryosurgery, Department Russian State University, Moscow, Russia, 1998
5. John H Greinwald Jr, Diane K Burke, Daniel J Bonthius, et al. An Update on the Treatment of Hemangiomas in Children
With Interferon Alfa-2a Arch Otolaryngol Head Neck Surg 1999;125:21-27
6. O’Donnell TMP, Devitt AT, Kutty S, et al. Recurrent congenital haemangiopericytoma in a Child. J Bone Joint
Surg [Br] 2001; 83-B : 269-72.
7. Beth A Drolet, Nancy B Esterly, Ilona J. Frieden. Hemangiomas in Children. N Engl J Med 1999; 341: 173-181
SARS
‘Masks seem to be essential for protection’
As concerns about SARS grow, reports that may help to further our understanding of the illness are presented in two Research letters. WH Seto and co-workers showed that masks helped to protect staff against infection in five Hong Kong hospitals, lending support to the notion that SARS is transmitted by droplets. Eugene B Wu and Joseph JY Sung describe a case that shows the importance of follow-up in patients with suspected SARS even if fever subsides, since there is often a break in pyrexia between the viraemic and lung-inflammatory stages. In a Commentary, Brian Tomlinson and Clive Cochrane relate their experiences in the hospital at the forefront of the SARS outbreak. The Editorial speculates on the potential public health problems if SARS affects world economies, and the News section rounds up the developments over the past week
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BMJ, 2003; 1485-86, 1519-20, 1525
