Biopsy-confirmed spontaneous resolution of orbital langerhans cell histiocytosis

A 17-year-old boy presented with a left upper lid swelling, headaches and diplopia. An orbital computerized tomography (CT) scan showed a mass in the left lacrimal fossa eroding bone and extending into the temporalis fossa and intracranially. An urgent biopsy without curettage was carried out and showed Langerhans cell histiocytosis. He was otherwise well and no other lesion was found. He was therefore observed and reviewed regularly. During follow-up it was noted that the mass was reducing in size. Five months after the initial biopsy a further procedure, involving curettage and an intralesional steroid, was carried out at a tertiary referral center. Histology of the material obtained showed no remaining evidence of Langerhans cell histiocytosis. Spontaneous resolution of orbital Langerhans cell histiocytosis has been described clinically and radiologically. This is the first case of spontaneous resolution to be confirmed histologically.     

Langerhans cell histiocytosis with periorbital cellulitis

PURPOSE: To report a case of Langerhans cell histiocytosis presenting as periorbital cellulitis. DESIGN: Interventional case report. METHODS: A 3-year-old girl presented with periorbital swelling of the right upper lid laterally of two days' duration. A history of recent eye trauma was reported. RESULTS: Computed tomography showed a soft tissue mass that eroded the frontozygomatic suture. The lesion was debulked through a lateral orbitotomy. Electron microscopy disclosed Birbeck granules. Immunohistochemistry stained positively for CD68 and CD1a antigenic determinants. CONCLUSIONS: Although rare, Langerhans cell histiocytosis can cause acute periorbital cellulitis in children. Trauma can induce an inflammatory response, allowing for earlier diagnosis of the orbital lesion.     

Congenital divided compound nevi of the eyelids

BACKGROUND: Congenital divided nevi of the eyelids are a rare melanocytic lesion. Only 30 patients are reported in literature. We report on a 6-year-old boy and a 41-year-old male with this lesion and give a short overview of the literature. CASE REPORTS: Patient 1: A 6-year-old boy presented with a pigmented divided nevus of the left eye involving the upper and lower lid naselly. The lesion had increased in size in the last one year. Otherwise the results of ophthalmological examination were unremarkable. The divided nevus was completely excised. Histopathological studies revealed a melanocytic compound nevus. Patient 2: A 41-year old male presented in our department with a divided pigmented nevus on his left eye involving the upper and lower lid laterally. The lesion had gradually increased since birth. Otherwise the results of ophthalmological examination were unremarkable. A subtotoal excision and debulking procedure was performed. The defect in the lower eyelid was covered using a transposition flap from the upper eyelid. Histopathological examination revealed a melanocytic compound nevus. CONCLUSION: Divided nevi of the eyelids may be disfiguring and include the possibility of deprivation amblyopia when reaching a certain size in childhood. Malignant changes of this lesion were not observed so far. Total excision or subtotal excision and a debulking procedure may be helpful.     

Caso clínico de histiocitosis de células de Langerhans presentándose como una celulitis orbitaria

Clinical case

A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Biopsy performed revealed the presence of Langerhans cell Histiocytosis. The lesion was surgically debulked and corticosteroids were used intra-operatively. The lesion responded to treatment.

Langerhans cell histiocytosis presenting as a limbal nodule in an adult patient

PURPOSE: To report a case of Langerhans cell histiocytosis presenting as a limbal nodule. DESIGN: Observational case report. METHOD: A 26-year-old man presented with a superior limbal mass. On clinical examination, an infiltrative/neoplastic lesion was suspected, and a differential diagnosis of limbal amyloidosis, limbal fibrous histiocytoma, lymphoma, and juvenile xanthogranuloma was made. There was no other associated ocular or systemic abnormality. An excision biopsy was performed. RESULTS: The histopathology and immunohistochemical staining examination established the diagnosis of limbal Langerhans cell histiocytosis. CONCLUSION: To the best of our knowledge, this is an unusual presentation of Langerhans cell histiocytosis, and although rare, Langerhans cell histiocytosis should be included in the differential diagnosis of a limbal mass.     

A 7th nerve palsy in a child with Langerhans histiocytosis

A 7-year-old girl developed an osteolytic soft tissue mass extending into the right orbital floor and maxillary sinus which was confirmed to be Langerhans cell histiocytosis. Partial lower motor 7th nerve palsy had developed, likely due-to a combination of local swelling from the tumour and the steroids.     

Langerhans Cell Histiocytosis of the Pituitary Gland Presenting as Unilateral Reversible Central Visual Loss and a Contralateral Junctional Scotoma

A 28-year-old woman developed Langerhans cell histiocytosis involving the hypothalamic-pituitary axis and presented with acute vision loss in the left eye, a junctional scotoma in the right eye, panhypopituitarism, and diabetes insipidus. Following endoscopic transphenoidal excision of the lesion, her vision improved in the affected eye and the junctional scotoma disappeared from the other eye. This is an unusual case of Langerhans cell histiocytosis of the hypothalamic-pituitary axis leading to a reversible anterior chiasmal (junctional) syndrome.     

Ungewöhnliche Präsentation einer Langerhans-Zell-Histiozytose

A 16-month-old male infant was presented with swelling of the left upper eye lid 4 weeks after a blunt orbital trauma. A prolonged hematoma was suspected and the child was discharged with an appointment 4 weeks later. However, the child was presented again with progressive swelling of the lid 10 days later. Magnetic resonance imaging (MRI) showed a tumor extending from the frontal bone to the anterior cranial fossa and into the orbit. An incisional biopsy led to the diagnosis of orbital Langerhans cell histiocytosis and systemic therapy led to complete remission of the tumor. Prolonged periorbital swelling must always prompt further diagnostics even when patients present with a history of trauma.     

Langerhans cell histiocytosis of the orbit 10 years after involvement at other sites

Langerhans cell histiocytosis within the orbit generally occurs in children and is marked by periorbital pain and swelling. Although these lesions respond well to minimal local curettage and concomitant corticosteroid injection, this disorder can be extremely aggressive and destructive. Recurrences usually occur within 12 months to 18 months after diagnosis. Herein, we discuss a patient who initially had mastoid and maxillary lesions and developed unilateral orbital involvement with periorbital pain and edema after a 10-year disease-free interval, and review other cases of late recurrence. The patient's symptoms resolved after curettage and injection of intralesional steroids. Clinicians should be aware that Langerhans cell histiocytosis may reappear at other sites, including the orbit, even after several years of quiescence.     

Therapieresistente Oberlidschwellung im Kindesalter

History

Swelling of the upper eyelid in childhood is caused by a variety of diseases and is very often generated by inflammation but orbital tumors should always be considered in the differential diagnostics.

Methods

We report about a 4-year-old girl with a drug-resistant swelling of the upper eyelid and ptosis of the right eye. This case report demonstrates the route from initial clinical examination to diagnosis and additionally reviews the current status of therapeutic options.

Results

After magnetic resonance imaging (MRI) and diagnostic excision, Langerhans cell histiocytosis (LCH) could be histologically proven. Visual acuity and levator muscle function improved from 0.5 to 1.25 and from 2 mm to 12 mm, respectively, by amblyopic prophylaxis and immunosuppressive therapy.

Conclusions

Persistent and drug-resistant swelling of the upper eyelid in childhood is also strongly suspicious for tumors. The suspicion of rare orbital tumors in children can be frequently substantiated by MRI. Biopsy and histological diagnosis are essential to plan adequate treatment and to estimate the prognosis. Particularly in Langerhans cell histiocytosis the methods of choice for over 10 years are specific immunochemical procedures (detection of protein s100 and CD1a).     

Rare Orbitocranial Tumour in an Adult

A 35-year-old lady presented with recurrent episodes of painful right upper lid swelling and ptosis of 6 months' duration. The symptoms would subside with oral antibiotics and painkillers. She had also been undergoing treatment for one year for multiple painful osteolytic lesions in the long bones and axial skeleton for which no definite diagnosis had been made, despite several radiological and serological investigations having been performed. Computed tomographic scan showed a soft tissue lesion in the superolateral part of the right orbit with erosion of the adjacent bone. Lacrimal gland biopsy showed non-specific inflammatory changes. She was referred for full work-up to exclude lymphoma and metastasis. Magnetic resonance imaging revealed an enhancing soft tissue mass in the right anterior cranial fossa eroding the lateral orbital wall and extending into the orbit. Another mass encased the superior sagittal sinus. Bone marrow biopsy showed near total replacement of marrow cells by a round cell tumour. Immunohistochemical studies revealed that the tumour cells expressed neuron specific enolase and chromogranin. The pathological findings suggested a diagnosis of neuroendocrine tumour though neuroblastoma could not be unequivocally ruled out. ¹³¹ I-metaiodobenzylguanidine scan showed no uptake. Radiologic studies suggested multiple bony metastases. No other site of primary tumour was found so there was a strong possibility of it being a primary orbito-cranial neurogenic tumour. Neuroblastomas and neuroendocrine tumours are very rare in adults. The orbit is an unusual site for a primary of this nature. In addition, it is quite difficult to make a specific diagnosis in a less differentiated tumour.     

Periocular keratoacanthoma: clinical features,pathology, and management

PURPOSE: To review the clinical features and results of surgical treatment of keratoacanthoma of the eyelids. DESIGN: Retrospective, interventional case series. PARTICIPANTS: Ten patients. METHODS: Chart review of all eyelid keratoacanthomas treated between 1992 and 2001. MAIN OUTCOME MEASURES: Adequate excision, recurrence rate, and complications. RESULTS: Patient ages ranged from 27 to 78 years, with a mean age of 59 years. Six patients were male and four were female. The lesion was found on the lower lid in five patients, upper lid in two, medial canthus in two, and lateral canthus in one. The maximum diameter of the lesion varied from 2 to 25 mm, with a mean of 7.2 mm. All lesions were treated by surgical excision, with frozen-section control of margins in five cases. All lesions were excised completely with clear resection margins, and there were no cases of recurrence. The only complication was a minor wound infection in one patient. Mean follow-up was 34.5 months. CONCLUSIONS: Because of the aggressive nature and uncertain relationship to squamous cell carcinoma, we recommend excision of periocular keratoacanthoma. Surgical excision of eyelid keratoacanthoma provides good results and a very low risk of recurrence. Frozen-section control of margins should be used in selected cases to ensure complete excision.     

Clinicopathological report: Mucinous carcinoma of the eyelid*

Background: Mucinous carcinoma of the skin is a rare tumour that may involve the peri-ocular region. Methods/Results: A case report is presented of a 73-year-old woman with a right upper lid tumour, initially diagnosed as a basal cell carcinoma. Excisional biopsy of the residual tumour revealed mucinous carcinoma. Re-examination of the original pathology proved to be mucinous carcinoma, originating in the eyelid skin. Further treatment involved wide local resection and reconstruction. Systemic investigations were undertaken to exclude the possibility of metastatic mucinous carcinoma. Conclusion: This case is presented to alert ophthalmologists to the occurrence of this rare tumour in the peri-ocular region, to highlight the importance of surgical excision with wide margins and the need for systemic investigation to exclude a primary malignancy in other sites, in particular the gastrointestinal system and breast.     

Clinicopathological report: mucinous carcinoma of the eyelid

BACKGROUND: Mucinous carcinoma of the skin is a rare tumour that may involve the peri-ocular region. METHODS/RESULTS: A case report is presented of a 73-year-old woman with a right upper lid tumour, initially diagnosed as a basal cell carcinoma. Excisional biopsy of the residual tumour revealed mucinous carcinoma. Re-examination of the original pathology proved to be mucinous carcinoma, originating in the eyelid skin. Further treatment involved wide local resection and reconstruction. Systemic investigations were undertaken to exclude the possibility of metastatic mucinous carcinoma. CONCLUSION: This case is presented to alert ophthalmologists to the occurrence of this tumour in the periocular region, to highlight the importance of surgical excision with wide margins and the need for systemic investigation to exclude a primary malignancy in other sites, in particular the gastrointestinal system and breast.     

Bilateral primary pseudotumoral palpebral amyloidosis. A case report

Ocular amyloidosis is infrequent, and the palpebral location is uncommon. It usually has a primary localized form, but can occur in systemic or familial amyloidosis. The diagnosis is mainly made by histopathology. Its treatment is surgical and the prognosis depends on clinical presentation and recurrences. We report the case of a 54-year-old woman with no medical history of the disease, who had bilateral isolated palpebral amyloidosis presenting as bilateral upper lid swelling with ptosis and corneal dystrophy. Diagnosis was confirmed by palpebral biopsy and the patient underwent excision of the involved lid tissue with reconstruction of the affected lid with cartilage autograft. Clinical progression showed improvement of the ptosis and the patient's comfort. Although rare, palpebral amyloidosis poses management problems, particularly in advanced cases, because of corneal complications and recurrence.     

Choroidal Neovascular Membrane Formation and Retinochoroidopathy in a Patient with Systemic Langerhans Cell Histiocytosis: A Case Report and Review of the Literature

We report a case of bilateral atrophic retinochoroidopathy with choroidal neovascular membrane (CNVM) formation in a patient with systemic Langerhans cell histiocytosis (LCH). A 35-year-old female, diagnosed with LCH at the age of 3, experienced an episode of acute vision loss in her right eye. Visual acuity was counting fingers. Dilated fundus exam and fluorescein angiography revealed the presence of CNVM along with bilateral widespread areas of chorioretinal atrophy. The patient underwent removal of CNVM with excellent postoperative visual acuity (20/25); however, indolent progression of her disease led to gradual deterioration of visual acuity (20/80 in the right eye and 20/320 in the left). This case shows that in contrast to previous reports, intraocular involvement of LCH does not need to be dramatic and clinically evident but it can acquire a chronic degenerative form. This report aims to raise awareness among ophthalmologists concerning the potential intraocular sequelae of LCH.Key Words: Langerhans cell histiocytosis, Choroidal neovascular membrane, Intraocular involvement     

Eosinophilic granuloma of the orbit with spontaneous healing

A 13-year-old boy presented with a 2-month history of swelling of the right upper eyelid and displacement of the right eye. Computed tomography (CT) showed a tumor in the right orbit and a large osteolytic defect of the orbital frontal bone. At surgery a yellowish-tan mass was found replacing the orbital roof and abutting the dura. Incisional biopsy established the diagnosis of eosinophilic granuloma. In the absence of further therapy, the mass resolved, and the bony defect completely reossified within 14 months. During more than 2 years of follow-up, there has been no evidence of local recurrence or systemic disease. This case demonstrates that eosinophilic granuloma may resolve spontaneously with healing of bony defects, thus eliminating the need for adjunctive resection or radiation therapy in selected patients.     

Sinus histiocytosis presenting as bilateral epibulbar masses

PURPOSE: To report a case of sinus histiocytosis presenting as multiple, bilateral epibulbar masses. METHODS: We examined a 4-year-old child with a 2-month history of gradually enlarging, salmon-colored epibulbar masses. Excisional biopsies and a systemic evaluation were performed. RESULTS: Systemic evaluation was normal; no lymphadenopathy was documented. Histopathologic evaluation disclosed features consistent with sinus histiocytosis. Flow cytometry and gene rearrangement studies confirmed the benign nature of the disease. No tumor recurrence was noted. CONCLUSION: Sinus histiocytosis may present as isolated epibulbar masses that may be treated with surgical excision.     

Eosinophilic granuloma of the orbit: report of two cases.

We present clinical, radiological, and histopathological findings and treatment results of two infants with orbital eosinophilic granuloma. Both patients presented at 1 year of age with unilateral proptosis, periorbital edema, and a superotemporally located, ill-defined orbital mass on computed tomographic (CT) imaging, with bone erosion in one patient. After subtotal excision, histopathological examination revealed eosinophilic granuloma, and the proptosis resolved with no sign of orbital tumor recurrence or systemic involvement at 12 and 23 months follow-up. Eosinophilic granuloma is a rare childhood orbital tumor that may or may not present with osteolytic change.