Benign fibrous histiocytoma of the conjunctiva in a patient with Human Immunodeficiency Virus: a case report

RR Nandkoomar^[1], María Carolina Salazar-Campos^[1], Juzer Surka^[1], Mirta Garcia Jardon^[2], Anderzj Stepien^[2]
(1) Departamento de Oftalmología, Universidad Walter Sisulu/Hospital Académico Nelson Mandela REPUBLICA SUDAFRICANA
(2) Walter Sisulu University REPUBLICA SUDAFRICANA

ABSTRACT

Purpose: To present a case of benign fibrous histiocytoma of the conjunctiva in a young adult with HIV

Design: A case report

Background: Benign fibrous histiocytoma is a common orbital tumour that occasionally affects the conjunctiva. It develops insidiously and can be locally infiltrating with a high recurrence rate. Can also undergo malignant transformation with a propensity to metastasize and can cause death.

Summary: A 38 year old HIV positive female patient presented with a conjunctival fibrous histiocytoma which was initially excised but then recurred with the recurrence being more aggressive than the primary tumour. The patient refused enucleation and the tumour was again excised. The patient is currently being monitored for recurrence.

Conclusion: It has not been established whether HIV increases the risk or incidence of developing fibrous histiocytoma or whether it increases the risk of malignant transformation.

Case Presentation    

Purpose: To present a case of benign fibrous histiocytoma of the conjunctiva in a young adult with HIV

Clinical History:

A 38 year old HIV positive female patient presented to us with a history of a mass growing in the right eye for 2 months. She did not report any associated symptoms. She was not on antiretroviral treatment and was generally in good health.

Clinical Examination:

General examination of the patient revealed no abnormalities.

Ophthalmic examination of the left eye was normal with a visual acuity of 1.5. Examination of the right eye revealed a 10mm x 8mm mass adjacent to the limbus at 3’o clock. The mass had a cauliflower- like appearance. Visual acuity in the right eye was 1.2 and the rest of the examination was unremarkable.

Management:

A provisional diagnosis of Squamous Cell Carcinoma was made and the patient was taken to theatre where an excisional biopsy was done and the specimen sent for histopathology.

The histopathology report diagnosed the tumour as a Benign Fibrous Histiocytoma (Figura 3 y 4) 

Clinical Course:

At the first follow up visit examination revealed a quiet eye with a vision of 1.2. The diagnosis was explained to the patient and the need for regular follow up visits was stressed because of the high incidence of tumour recurrence. The patient however defaulted on follow up and next presented to us 7 months later with tumour recurrence.

On examination the patient now had a large fungating mass (fig.1) covering the nasal half of the cornea and extending into the nasal halves of the superior and inferior fornices as well as medially up to the canthus. The mass was associated with significant pain and displayed areas of necrosis as well as areas of surface vascularization. There was marked limitation of elevation, depression and adduction. Visual acuity was reduced to 0.3.

A CT scan was done and showed a soft tissue lesion adherent to the sclera on the medial aspect of the globe but no extension beyond the orbit.

Blood investigations were normal and the CD4 count was 455 x 10^6/l.

The patient was not on any anti-retroviral therapy.

In light of the aggressive presentation, a differential diagnosis included the following:

1)    Locally aggressive recurrence of the original fibrous histiocytoma

2)    Malignant transformation of the tumour

3)    A new malignant tumour such as a lymphoma or amelanotic melanoma.

The patient was counselled on the possible benefit of having an enucleation but she refused. For this reason, she was then taken to theatre and had an excisional biopsy done but complete excision was not possible. Tumour debulking was performed and specimens sent for histology.

The pathologist at Nelson Mandela Academic Hospital reported that the tumour was composed of areas of fibrosis and hyalinization surrounded by cellular infiltrate of macrophages, foam cells, multinucleated giant cells and lymphocytes. A few larger cells with atypical and prominent nucleoli were present.

Immunohistochemistry studies showed strongly positive CD68, focally positive Factor 13A, CD34 was positive only in blood vessels and S100 negative.

A differential diagnosis included xanthogranuloma, fibrous histiocytoma, malignant lymphoma and Hodgkin’s lymphoma and the specimen was sent to NHLS laboratories at Tygerberg for further evaluation.

A final diagnosis of an incompletely excised atypical benign fibrous histiocytoma was made by the pathologists (Figura 3 y 4).

The patient is currently being followed up at our department. At her last visit she had a visual acuity of 0.4 in the right eye (fig.2), with a leucomatous corneal opacity of the nasal half of the cornea and involving part of the visual axis. Residual tumour tissue is still present over the nasal conjunctiva. Eye movements have improved but residual deficits in adduction remain. The patient is still not on antiretrovirals (CD4 count 400 x 10^6/l) and refuses any further surgical intervention.

[[Fig. 1]] - Preoperative view of the lesion. Macroscopic view of the lesion.

[[Fig. 2]] - Post operative view after surgery

Figura 3 - Primera biopsia realizada.

Figura 4 -

Figura 5 -

Figura 6 -

Comments    

Fibrous Histiocytoma is a spindle cell tumour that originates from fibroblasts and histiocytic cells and is one of the commonly occurring lesions in the adult orbit.

Fibrous histiocytoma of the conjunctiva, however, is a rare occurrence. Shield’s et al conducted a review of 1643 conjunctival tumours found that fibrous histiocytoma represented only 0.2% of all tumours. In reviewing all patients who presented with conjunctival tumours at Will’s Eye Hospital between 1974 and 2005, Kim et al detected only 6 cases of fibrous histiocytoma. In a further review of the literature Kim et al found that only 29 cases of conjunctival fibrous histiocytoma reported since 1968. This included their 6 cases at Will’s Eye Hospital.

The mean age of presentation was 39 years (range <1year to 78 years) with no gender predilection (13 females and 16 males).

The mean duration of symptoms prior to diagnosis was 8 months (range 1 week to 36 months). 27 cases were unilateral (11 OD and 16 OS) and 2 bilateral.

74% of cases were located at the corneoscleral limbus and 22% at the cornea. The mean tumour basal diameter was 8mm (range 2-19mm) and mean thickness 5mm (range 2-9mm).

Typical presentation is a firm red to tan mass arising from the conjunctival stroma near the corneoscleral limbus. Fine feeder vessels over the tumour surface may be visible.

Histologically, it has 2 primary elements viz spindle shaped fibroblasts and rounded histiocytic cells arranged in an interwoven pattern or in a storiform pattern radiating from central focal points of condensed collagen.

Immunohistochemistry, although not specific enough to distinguish fibrous histiocytoma from other mesenchymal lesions, is capable of excluding other spindle cell tumours.

Although these tumours are mainly benign they are locally aggressive with a high propensity for recurrence especially in anatomically restricted sites like the conjunctiva and orbit.

Malignant transformation is rare, with only 9 of the 29 reported cases showing transformation.

The recommended treatment is complete surgical excision with tumour free margins. Local recurrence, orbital invasion and metastases typically occur following incomplete excision. In recurrent cases, repeat excision is advised. If tumour growth is extensive and complete excision with tumour free margins is not possible, then enucleation or exenteration is warranted. In malignant cases radiotherapy may be a useful adjuvant to surgical excision to improve local control.

A literature search revealed no reported cases of fibrous histiocytoma of the conjunctiva in HIV positive patients.

Conclusion    

It has not been established whether HIV increases the risk or incidence of developing fibrous histiocytoma or whether it increases the risk of malignant transformation. The patient has not come back yet for following up.

Bibliografía    

Comentarios

- NELSON BUSTAMANTE SIGARROA - REPUBLICA SUDAFRICANA  (15/11/2009 5:53:22)

Excelente trabajo, como escribio alguien en un comentario, "en este congreso se aprende" a lo que yo agrego no solo los patologos.

Nuevamante felictaciones Dr. Nandkoomar

Prof. Nelson Bustamante Sigarroa
Africa del Sur

- Rafael Martínez Girón - ESPAÑA  (16/11/2009 22:54:13)

Congratulations for this interesting case report. I think that papers like this will confirm the important role of micro-organisms in the aetiology of tumours.
Kind regards from Asturias.

- Emilio Mayayo Artal - ESPAÑA  (24/11/2009 23:53:58)

Congratulations for the case.