Indian Journal of Surgery Medknow Publications on behalf of Association of Surgeons of India ISSN: 0972-2068 Vol. 65, Num. 5, 2003, pp. 438-440

Indian Journal of Surgery, Vol. 65, No. 5, Sept-Oct, 2003, pp. 438-440

Case Report

Lethal midline granuloma - epitheloid angiosarcoma face

Leo Francis Tauro, Sathyamoorthy Aithala, Leo Theobald Menezes, Nandakishore B.*

Department of General Surgery and *Dermatology, Fr. Muller's Medical College Hospital,Kankanady, Mangalore 575002. D.K., Karnataka.
Address for correspondence: Dr. Leo Francis Tauro, Asst. Professor, Department of General Surgery, Fr. Muller's Medical College Hospital, Kankanady, Mangalore 575002. D. K. Karnataka. E-mail:drlftauro@rediffmail.com

Paper Received: August 2000. Paper Accepted: January 2002. Source of Support: Nil.

How to cite this article: Tauro LF, Aithala S, Menezes LT. Lethal midline granuloma - epitheloid angiosarcoma face. Indian J Surg 2003;65:438-40.

Code Number: is03089

ABSTRACT

Angiocentric immunoproliferative and locally destructive lesions involving the midface, nasal cavity, paranasal sinuses and / or the upper aerodigestive tract have been referred to in the past as lethal midline granuloma. This includes neoplastic, infective, vasculitic and idiopathic midline destructive disease. We report such a case of neoplastic origin which presented with necrotising lesion of the mid-face involving the right orbit and the nasal bridge. Histologically, it was proved to be angiosarcoma and was treated with chemotherapy.

Key Words: Lethal midline granuloma, Angiosarcoma.

INTRODUCTION

Lethal midline granuloma (LMG) describes lesions of the mid-face which are characterised by progressive and often fatal ulceration and destruction of the upper airway involving the nose, the paranasal sinuses, the palate, orbit and the soft tissues of the face. Four entities are included under this term: idiopathic midline destructive disease (IMDD), polymorphic reticulosis, non-Hodgkin's lymphoma and Wegener's granulomatosis.^1,2 We report a case of necrotising lesion of the mid-face involving the right orbit and the nasal bridge.

CASE REPORT

A 14-year-old boy presented with 1½ years history of painful ulcerative lesion over the face. The lesion started following a nail prick injury, as multiple papules at the lateral canthus of the right eye, which later ulcerated. The ulcer had been spreading since 9 months. General and systemic examination including that of the respiratory system revealed no abnormality. Local examination showed a destructive, ulcerative lesion involving the entire right orbital region, bridge of the nose and crossing the midline. The lesion had sloping edges, slough and unhealthy granulation at the floor, and was indurated as well as tender. The base was formed by underlying bone. The right eyeball was completely destroyed. There was no bleeding on touch and sero purulent discharge was present from the ulcer (Figure 1). Our clinical differential diagnosis was cancrum orbitalis and nasalis, LMG and actinomycosis face.

Routine haematological and biochemical investigations were normal except an ESR of 65 mm/hr. Staphylococcus aureus and E. Coli were cultured from the wound. Skull X-ray showed orbital bone destruction. Chest x-ray was normal. CT scan showed extensive bone destruction involving the medial wall and floor of the orbit, the orbital fissure and sphenoid bone. There was a small extradural collection in the right frontal region and osteomyelitis of the right frontal bone. All these features pointed to an inflammatory pathology. Biopsy from the edge and base of the ulcer showed epitheloid angiosarcoma (Figure 2). Since the growth was extensive and inoperable, the patient was treated with chemo and radiotherapy.

DISCUSSION

Angiocentric immunoproliferative lesions involving the mid-face, nasal cavity, paranasal sinuses, and / or upper aerodigestive tract have been referred to in the past as LMG.³ When neoplastic, infective, vasculitic causes have been excluded, the diagnosis of IMDD is possible.⁴

Angiosarcoma of the skin is a rare malignant tumour which occurs in both sexes. Often, in elderly individuals it occurs either in a chronic lymphoedematous limb, e.g. in an arm after a radical mastectomy and radiotherapy for breast cancer or in the head and neck region in the scalp.⁵ Some of these lesions have been associated with foreign material introduced into the body either iatrogenically or accidentally.⁶ The tumour presents as red plaques and nodules which may be very extensive and which tend to ulcerate. Microscopically, the tumour consists of an anastomosing network of vascular channels, lined by atypical endothelial cells, dissecting through the dermal collagen. Areas of poorly differentiated solid spindle cell appearance may be present.^5,6

The prognosis is very poor. These lesions often metastasise to the regional lymph node and distant sites, but death may occur before development of metastses in head and neck tumours as a result of extensive local destruction.

REFERENCES

1. Szyfter W, Wierzbicka M. On classification of changes in necrotising lesions of the midface; Otolaryngol Pol 1996;50:207-20.
2. Graper RG, Orenstein HH, Rohrich RJ, Byrd HS, Rich BK. Idiopathic Midline granuloma - current classification and management controversies. Ann Plast Surg 1996;37:532-7.
3. Irwin M. Freedberg, Arthur Z. Eisen, Klaus Wolff, K. Frank Austen, Stephen I Kartz, et al. Thomas B. Fitzpatrick et. al. Inflammatory diseases that simulate lymphoma; Fitzpatrick's Dermatology in General Medicine, 5th edn. MC Graw Hill, New York; 1971. Vol I. pp. 1271-2.
4. Barker THW, Hosni AA : Idiopathic midline destructive disease - does it exists ? J Laryngol Otol 1998;112:307-9.
5. Roddie MN. Mac sween, Keith Whaley. The Skin - Muir's Text Book of Pathology, 13^th edn. EL BS; 1997. pp. 1139-40.
6. Ramzi S. Cotran, Vinay Kumar, Tucker Collins. Blood Vessels - Robbins Pathologic Basis of Disease. 6th edn. WB Saunder Company; 1999. pp. 537- 8.

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