British Journal of Ophthalmology, 1989, 73, 373-375

Adult periorbital fibromatosisCATHERINE N SMOOT, GREGORY B KROHEL, AND RICHARD S SMITH

From the Department of Ophthalmology, Albany Medical College, New York

SUMMARY Three patients aged 15 to 40 years with fibromatosis of the periorbital region werestudied. A history of acute onset, preceding trauma, and pain associated with a palpable mass weretypical features. Rapid recurrence followed surgical excision. Misdiagnosis is common in thisbenign disease and may lead to unnecessary examinations for malignancy. Surgical excision or

debulking of these masses is indicated for diagnosis and pain relief, but long-term follow-upindicates that these lesions will often spontaneously resolve with time.

Benign acute recurrent fibromatosis is a fibroblasticproliferation that lies in a range between normalrepair with scar formation and fibrosarcoma. Reportshave suggested that trauma may play a part in thedevelopment of this disease,' though other reportsdispute this connection.24 These fibrous masseshave no definable margins and may locally invadebone and striated muscle. They do not tend tometastasise. 56 The pathological diagnosis may beconfusing and malignancy is often initially suspected.

Juvenile fibromatosis is a well described clinicalentity that occurs throughout the body, though theabdomen appears to be the most frequent site.'Fibromatosis of the periorbital region has previouslybeen described in children.4 Clinical characterisationof this entity in adults has not been described in theophthalmic literature. We recently treated threewomen with recurrent periorbital fibromatosis.

Case reports

A 24-year-old nursing student first presented toAlbany Medical Center Hospital (AMCH) in January1984 with a corneal ulcer refractory to treatment. Shedeveloped ecchymosis, swelling, and warmth of theleft infraorbital region while in the hospital. Thisappeared to respond to intravenous antibiotictherapy, though a small nodule remained over theleft premalar area. In April the patient was noted tohave increasing ecchymosis of the left lower lid andpremalar region. An excisional biopsy via a subciliaryeyelid incision revealed diffuse fibrotic tissue over thepremalar area and extending into periosteum. The

Correspondence to Gregory B Krohel, MD, Albany MedicalCollege, Department of Ophthalmology, Albany, New York 12208,USA.

initial pathological interpretation was rhabdomyo-sarcoma and an oncology specialist was consulted.Re-examination of the specimen suggested a reactivemyositis.One month later the patient presented with a

painful ecchymotic mass and a small eschar in thepremalar region. A second biopsy was obtained and adiagnosis of fibromatosis was made. In Octoberof 1984 the eschar enlarged and she underwentdrainage of an abscess over the left malar eminence.Manipulation of the wound site by the patient wassuspected and she was referred to the psychiatricservice for evaluation of probable Munchausen'ssyndrome.7 Her clinical course improved slowlyunder psychiatric observation. No recurrence of herproblem has been noted over the past two years.

CASE 2A 15-year-old female presented to AMCH in May1981 with a history of spontaneous left malar andinfraorbital ecchymosis. It was later' thought shemight have suffered injury from her boyfriend. Apainful, palpable, ecchymotic mass was noted alongthe left inferior rim (Fig. 1). A subciliary excisionalbiopsy was performed and initially interpreted asnodular fasciitis. Further review and consultationwith the Armed Forces Institute of Pathologyconfirmed the diagnosis of benign fibromatosis. Thepatient experienced several recurrent episodes ofpainful ecchymosis, with regrowth of the mass. Arepeat anterior orbitotomy performed six monthslater confirmed the diagnosis of fibromatosis. Overthe next three years she had several episodes ofpainful swelling and ecchymosis lasting one to twoweeks. Each recurrence tended to be less severe andresolved spontaneously. Further surgery was notnecessary.

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CatherineN Smoot, Gregory B Krohel, and Richard S Smith

Fig. 1 Ecchymotic mass over the left infraorbital and malararea secondary tofibromatosis.

CASE 3A 40-year-old nurse was accidentally struck force-fully in the left infraorbital area by a patient's headin November 1986. This resulted in persistent,painful infraorbital swelling over the left malararea accompanied by a dimpled contracture. Twomonths later a fibrotic mass was removed by anteriororbitotomy through a subciliary incision. Patho-logical examination confirmed the clinical diagnosisof fibromatosis. Two weeks postoperatively shepresented with a recurrent painful mass. The decisionwas made to institute massage therapy with vitamin Ecream while observing the clinical course. Thedimpling and pain had decreased four months later.A small pea sized nodule remained. By September1987 the dimpling and pain had resolved, and only asmall soft nodule over the left lateral premalar arearemained. No further surgery is planned.

Discussion

Fibromatosis in adults and children has beenpreviously described in various sites of the bodyincluding the periorbital region. I 4 8 9Our three casesof adult periorbital fibromatosis had several features

in common. Two patients had a definite history oftrauma immediately preceding the onset of the lesionand trauma was strongly suggested in the third case.Trauma has been mentioned as an aetiological agentin some cases of fibromatosis,' though five of sixpatients previously described with juvenile peri-orbital fibromatosis had no history of trauma.4Despite the ecchymotic appearance of these lesionshaemosiderin was observed in only one of our threepatients.

All three patients in our series were women. Afemale preponderance had been noted previously,suggesting a hormonal influence in the developmentof fibromatosis.8The prevalence of pain with fibromatosis remains

uncertain. Conley et al.3 noted an absence of pain inseven patients with periorbital lesions, while twopatients with periorbital lesions in another series hadassociated pain.8 The onset of symptoms was acuteand painful in the three patients we treated. Thiscontrasts with the cases of juvenile periorbital fibro-matosis previously reported in the ophthalmicliterature which were described as firm, painlesslumps, with no mention of acute onset.4

Misdiagnosis of this benign tumour can occur.9One of our patients underwent examination forsuspected rhabdomyosarcoma. The other mis-diagnoses in our patients were reactive myositis andnodular fasciitis.A surgical approach via a subciliary incision in

all three patients yielded excellent postoperativecosmesis (Fig. 2). A firm, whitish rubbery massextending from subcutaneous tissue to periosteumwas observed in all cases. Integrity of the surrounding

Fig. 2 Postoperative appearance ofpatient in Fig. I with agood cosmetic result. The patient had several recurrencesfollowing surgery.

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Adultperiorbitalfibromatosis

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Fig. 3 A focus ofperivascular fibrous tissue with Fig. 4 Intracellular haemosiderin deposits are presentlymphocytic infiltration (arrow) consistent with fibromatosis. (arrows). Haemosiderin was noted in only one ofthree(Haematoxylin and eosin x 150). patients despite the ecchymotic appearance ofall three

lesions. (Haematoxylin and eosin, x 60).

bony structures was preserved in two cases, thoughlocal bony invasion similar to that previouslyreported was observed in one patient.3

Histopathologically, fibromatosis is a proliferationof fibroblasts with interstitial collagen and raremitotic activity.9 This differs from fibrosarcomawhich has less interstitial collagen and more mitoticactivity.'0 Hidayat and Font4 microscopically noted aleiomyomatous appearance of the tumours in threeof the juvenile patients they reported, a picturesimilar to that of congenital generalised fibromatosis.Our pathology sections showed dense perivascularcollections of fibrous tissue with frequent foci oflymphocytes in all specimens (Fig. 3). The fibroblasticcells varied in shape from spindle to stellate, whilenuclei differed in their size and degree of basophilicstaining. Haemosiderin was noted in the pathologicalspecimens in one case (Fig. 4). Electron micro-graphic (EM) studies confirmed the fibroblasticnature of the lesion in one patient. Character-istic features shown included a proliferation ofmesenchymal fibroblasts with an increase in roughcytoplasmic reticulum and the presence of inter-cellular fibrin and collagen.The treatment employed was local surgical

excision of the lesion. The therapeutic value ofsteroids,6' radiation,3' 12 or chemotherapy'3 remainsinconclusive. Despite good surgical results all ourpatients had recurrences. We decided to reoperateonly if the pain could not be controlled medically.Two of our three patients required further surgery forthat reason, while one was successfully managed withtime and massage after the initial resection.

In summary, benign acute idiopathic fibromatosisshould be considered in the differential diagnosis of

acute, tender, palpable periorbital lesions in adults.A careful history may reveal a preceding traumaticincident. Surgical excision affords local pain reliefand cosmetic improvement, but recurrences arecommon.

This work was supported in part by an unrestricted grant fromResearch to Prevent Blindness, Inc.

References

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fibromatosis. Cancer 1958; 11: 627-39.3 Conley J, Healey WV, Stout AP. Fibromatosis of the head and

neck. Am J Surg 1966; 112: 609-14.4 Hidayat AA, Font RL. Juvenile fibromatosis of the periorbital

region and eyelid. Arch Ophthalmol 1980; 98: 280-5.5 Yao-Shi-Fu, Perzin KH. Nonepithelial tumors of the nasal

cavity, paranasal sinuses and nasopharynx: a clinicopathologicstudy: VI. Fibrous tissue tumours (fibroma, fibromatosis,fibrosarcoma). Cancer 1976; 37: 2912-28.

6 Prior JT, Sisson BJ. Dermal and fascial fibromatosis. Ann Surg1954; 139: 453-67..

7 Rosenberg PN, Krohel GB, Webb RM, Hepler RS. OcularMunchausen's syndrome. Ophthalmology 1986; 93: 1120-3.

8 Musgrove. JE, McDonald JR. Extra-abdominal desmoidtumors. Arch Pathol 1948; 45: 513-40.

9 Mackenzie DH. The fibromatosis: a clinicopathological concept.Br Med J 1972; iv: 277-81.

10 Font RL. Eyelids and lacrimal drainage system. In: SpencerWH, ed. Ophthalmic pathology. Philadelphia: Saunders, 1986:3: 2294-6, 2578-80.

11 Benninghoff D, Robbins R. The nature and treatment ofdesmoid tumors. AJR 1964; 91: 132-7.

12 Hill DR, Newman H, Philips TL. Radiation therapy of desmoidtumors. AJR 1973; 117: 84-9.

13 Stein R. Chemotherapeutic response in fibromatosis of the neck.J Pediatr 1977; 90: 482-3.

Acceptedfor publication 16 August 1988.

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