Malignant orbital disease in children

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Although very rare, the very aggressive malignancies of rhabdomyosarcoma or neuroblastoma tend to present under the age of 10 years, the acute haematological malignancies within the first two decades and primary lacrimal gland malignancy has a peak incidence in the fourth decade.


Rhabdomyosarcoma, with a peak incidence at age 7, is the commonest primary orbital malignancy of childhood and arises from pleuripotent mesenchyme that normally differentiates into striated muscle cells. Although rhabdomyosarcoma classically presents with signs of acute orbital cellulitis (Figure 13.1a), in some cases it is more insidious and mimics a benign process; a high index of suspicion is required for any unilateral orbital disease in childhood. At this age the main differential diagnosis for a rapidly growing tumour mass is a deep orbital capillary haemangioma, although children with haemangiomas will often have other cutaneous vascular lesions.

The tumour mass may be located anywhere in the orbital soft tissues, most commonly in the supero-medial quadrant, and typically does not arise in the extraocular muscles. Orbital imaging will usually demonstrate a fairly well defined, round mass arising within the orbital fat and flattening the globe (Figure 13.1b),

Figure 13.1 Childhood rhabdomyosarcoma may present as a rapidly growing orbital mass (a) or with inflammatory signs; (b) the rapidly progressive tumour may compress the globe and typically is not associated with muscle.

Figure 13.1 Childhood rhabdomyosarcoma may present as a rapidly growing orbital mass (a) or with inflammatory signs; (b) the rapidly progressive tumour may compress the globe and typically is not associated with muscle.

the tumour showing moderate contrast enhancement. Expansion of the adjacent thin childhood orbital bones is fairly common, but calcification of the tumour is rare.

Doppler ultrasonography may be helpful in differentiating capillary haemangiomas from rhabdomyosarcomas, the haemangiomas showing marked vascularity with very high flow-rates.

Urgent incisional biopsy, using an anterior transcutaneous or transconjunctival approach (Chapter 12), is required to confirm the diagnosis, although macroscopic excision may be possible for well-defined small tumours. On confirmation of diagnosis, a systemic evaluation, including whole-body CT scan and bone marrow biopsy, is required to look for metastatic disease.

The commonest variant of the tumour is the embryonal type, the alveolar is clinically aggressive with a bad prognosis, and the pleomorphic variant (the rarest) has the best prognosis. The 5-year survival is greater than 90% with local radiotherapy and adjuvant chemotherapy as the mainstay of treatment, although local resection of residual tumour (or orbital exenteration) may be needed in a few cases.

Long-term side-effects of orbital radiotherapy include cataract, dry eye with secondary corneal scarring, loss of skin appendages (lashes and brow hair), atrophy of orbital fat and, if performed in infancy, retardation of orbital bone growth. There is also a risk of late radiation-induced orbital malignancy, such as fibrosarcoma and osteosarcoma, and there may be an increased propensity to certain other primary tumours in adulthood.

Figure 13.2 Neuroblastoma metastatic to the orbital rim in an infant.

rhabdomyosarcoma. Another childhood malignancy that may present with orbital inflammatory signs is acute myeloid leukaemia (Figure 13.3a); this is also known as "chloroma", the tumour tissue turning green on exposure to air (Figure 13.3b). Langerhans cell histiocytosis (of which there


Other malignancies

Neuroblastoma may present as rapidly progressive metastasis within the orbital soft tissues or bone (Figure 13.2), the clinical presentation being very similar to

Figure 13.3 (a) Acute myeloid leukaemia presenting with persistent orbital cellulitis; (b) the tumour may be termed chloroma because the tissue turns green in air.

are three main variants) is a proliferative disease of Langerhans cells that may be malignant, although the variant found most commonly in children - eosinophilic granuloma - verges on a benign proliferation and is readily treated.

All of these childhood tumours require urgent biopsy, systemic investigation and chemotherapy with, in some cases, radiotherapy. Although prognosis for vision with most of the haematological malignancies is generally good there is a significant mortality, depending on pre-chemotherapy disease staging.

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