Neoadjuvant chemotherapy for invasive squamous cell carcinoma of the conjunctiva: A case report

A 40-year-old male presented with an orbital extension of conjunctival squamous cell carcinoma (SCC). The orbital mass was seen protruding outward from the left palpebral fissure overhanging the lower eyelid, completely obscuring the globe and lower lid. The patient gave a history of excision biopsy, which was histopathologically diagnosed as ocular surface squamous neoplasia. He also gave a history of tumor recurrence, which gradually progressed to assume the form of the presently visible orbital mass. Computed tomography of the orbits showed the mass extending into the left orbit causing superior displacement of the globe. After a negative locoregional and systemic metastatic screening, neoadjuvant intravenous systemic chemotherapy with cisplatin and 5-fluorouracil were initiated in an attempt to reduce the size of the tumor. Three cycles of tri-weekly chemotherapy resulted in a significant reduction of the orbital tumor size with the globe and the lower lid being visible, thus making a lid-sparing orbital exenteration possible. The patient subsequently underwent an orbital exenteration and at 6-month follow-up, the patient was free from local and regional disease. To our knowledge, this is the first reported case where systemic neoadjuvant chemotherapy has been used to reduce the size of invasive SCC with orbital extension, thereby permitting a lid-sparing orbital exenteration.     

Recurrent and residual sebaceous carcinoma after Mohs' excision of the primary lesion

We reviewed the histories of three patients with sebaceous carcinoma (two of the eyelid and one of the caruncle) who had had their tumors excised by surgeons experienced in the Mohs' technique. In each patient the tumor was considered totally excised. In one patient, residual intraepithelial pagetoid sebaceous carcinoma of the eyelid was detected incidentally when a reparative flap was placed. In the other two patients, invasive sebaceous carcinoma recurred, necessitating orbital exenteration; one patient has regional lymph node metastases. Because of the difficulty in detecting pagetoid spread on frozen sections and the multicentric nature of sebaceous carcinoma, we advocate managing these tumors by wide local excision, checking margins with high-quality permanent sections.     

Surgical management of squamous cell carcinoma of the lid

A series of 12 patients with eyelid squamous cell carcinoma was studied retrospectively with emphasis on the clinical presentation, management, and results. Of the five cases that constituted our personal experience, surgical excision with frozen-section control was an efficacious mode of treatment. In the overall series, there were no cases of regional lymph node metastasis and no tumor deaths. One patient required orbital exenteration. The methods of surgical extirpation of the tumor with frozen-section control, as advocated by the authors, are similar to those used in the management of basal cell carcinoma and other eyelid neoplasms. Because of the potential lethality of squamous cell carcinoma, a wider excision than that usually performed for basal cell carcinoma is recommended, as is close postoperative follow-up.     

Desmoplastic spindle-cell melanoma of the eyelid with orbital invasion

PURPOSE: To describe the clinicopathologic features of a patient with a spindle-cell melanoma of the eyelid that exhibited orbital invasion. METHODS: Case report. RESULTS: A slowly enlarging mass developed in the eyelid of a 50-year-old woman. Excision of the mass showed desmoplastic spindle-cell melanoma. The tumor recurred in the orbit, and an exenteration was performed. A second recurrence 7 months later was treated with radiation therapy. CONCLUSIONS: There is a continuum of spindle-cell to desmoplastic melanoma. The prognosis of patients with orbital invasion of melanoma is poor, and adjuvant treatment including radiation and chemotherapy has little benefit.     

Meibomian gland adenocarcinoma with regional lymph node metastasis

Meibomian gland carcinoma of the eyelid is a common neoplasm which clinically simulates a benign condition of chalazion. Although growing slowly, metastasis to regional lymph nodes is frequent, at a rate of 17-28% in this country. Eleven (11) cases of Meibomian gland carcinoma with regional lymph node metastasis are reported, and the therapeutic modalities have been tailored to suit the individual cases, including full-thickness local excision, orbital exenteration, radical cervical dissection, and superficial parotidectomy.     

Experience with eyelid-sparing orbital exenteration: the 2000 Tullos O. Coston Lecture

PURPOSE: To provide data on types of lesions that require orbital exenteration and discuss our experience with exenteration, emphasizing the eyelid-sparing technique. METHODS: The records were reviewed on all patients who underwent orbital exenteration by the senior author and his colleagues. The underlying disease, method of exenteration, healing times, complications, cosmetic outcome, local tumor control, and patient survival were analyzed. RESULTS: Of 56 orbital exenterations, 47 were done by eyelid-sparing technique and 9 by eyelid-sacrificing technique. The 9 tumors of eyelid origin that required exenteration included basal cell carcinoma (4), sebaceous gland carcinoma (3), squamous cell carcinoma (1), and melanoma (1). Four of the eyelid lesions were managed by the eyelid-sparing technique and 5 by the eyelid-sacrificing method. The 24 tumors of conjunctival origin included melanoma (20) and squamous cell carcinoma (4). The eyelid-sparing method was used in 22 of the 24 cases. The 10 primary orbital lesions included melanoma (3), malignant lacrimal gland tumor (3), a rhabdoid tumor, meningioma, Wegener's granulomatosis, and aspergillosis. The eyelid-sparing method was used in 8 of the 10 cases. Among intraocular tumors were uveal melanomas (10) and retinoblastoma (1). The eyelid sparing method was used in all 11. With the eyelid-sparing technique, the mean time from exenteration to wound healing and readiness for a prosthesis was 4 weeks (median, 2 weeks; range, 2 to 24 weeks). With the eyelid-sacrificing technique, the mean time was 68 weeks (median, 34 weeks; range, 2 to 340 weeks). Complications were few and cosmetic results were satisfactory. Local tumor control with exenteration was achieved in 53 of 56 cases, but metastasis occurred in 11 of 20 cases of conjunctival melanoma (55%) and in 6 of 10 patients with uveal melanoma (60%). Metastasis has developed in none of the 3 patients with malignant epithelial tumors of lacrimal gland origin. CONCLUSIONS: Orbital exenteration is an appropriate procedure for orbital involvement by unresectable malignant tumors of the eyelids, conjunctiva, intraocular structures, and orbit. In most of these cases, including about half of tumors originating in the eyelids, an eyelid-sparing exenteration can be used to achieve more rapid healing.     

Orbital reconstruction after eyelid- and conjunctiva-sparing orbital exenteration

PURPOSE: To describe the technique and results of eyelid- and conjunctiva-sparing orbital exenteration with temporalis transfer and dermis-fat graft. METHODS: Five patients who underwent this procedure were studied, based on retrospective chart review. Schematic drawings were produced to illustrate the surgical technique. RESULTS: The study included three female and two male patients with an age range of 7 to 76 years. Two patients required surgery for invasive optic nerve sheath meningioma, one for hemangiopericytoma, and the fourth for mesenchymal chondrosarcoma. The last patient had eyelid-sparing exenteration performed more than 20 years earlier for retinoblastoma and underwent a similar reconstruction. One case was complicated by infection and graft atrophy. The remaining four cases retained orbital volume and eyelid structures with a reasonable cosmetic outcome. Average follow-up period was 21 months, during which one patient had development of intracranial meningioma at the proximal end of the optic canal. CONCLUSIONS: Eyelid- and conjunctiva-sparing orbital exenteration with temporalis transfer and dermis-fat graft is a useful technique that minimizes the usual deformity and achieves good results. Case selection is emphasized, as this technique is mainly reserved for histopathologically benign orbital lesions that exhibit local aggressive behavior and for malignant lesions only if there is no eyelid, lacrimal gland, or orbital fissure involvement nor significant conjunctival or deep extension of an intraocular tumor.     

Reconstruction of orbital exenteration defects with bilobed forehead flap

To describe the use of bilobed forehead flap for reconstruction of orbital exenteration defect. The medical records of 2 patients in whom orbital exenteration defect repair was performed with bilobed forehead flap were reviewed. In both patients (male, ages 74 and 65 years), extended exenteration was performed because of basal cell carcinoma infiltrating the upper and lower eyelids and orbit. One patient had a history of multiple eyelid surgeries and periorbital radiotherapy. In the other, the tumor also involved the maxillary and ethmoid sinuses and nasal dorsum. The bilobed flap was combined with a cheek advancement flap in 1 patient. The excisional defect could be primarily covered in both patients. In 1 patient, a skin graft was needed to cover the forehead donor area defect. In both patients, transient, distal flap ischemia developed after surgery and, in 1 patient, eyebrow malposition required surgical correction at the late period. No other complication developed during follow-up (18 and 26 months). The bilobed forehead flap can be effectively used to reconstruct total or extended orbital exenteration defects.     

Adenocarcinoma of the lacrimal gland with sebaceous differentiation

A case of metastasizing adenocarcinoma of the lacrimal gland with sebaceous differentiation is described, which probably originated in a benign mixed tumour (BMT), with infiltration of the orbital muscles, the bony orbital wall, the neck lymph nodes, and the parotid gland. The patient was a 69-year-old male with a proptosis that had persisted for 31 years. An orbital exenteration was performed. Death occurred due to cerebral tumour infiltration. In view of the sebaceous pattern meibomian gland carcinoma has to be ruled out in the differential diagnosis.     

眼睑恶性肿瘤的临床分析

目的分析眼睑恶性肿瘤的临床病理类型和治疗方法。方法对74例眼睑恶性肿瘤患者进行病例回顾性分析。其中,男性35人,女性39人,肿瘤部位:右眼38人,左眼36人,上睑25人,下睑49人,年龄28岁～84岁,平均60.74岁。病理诊断示:基底细胞癌39例,睑板腺癌22例,鳞状上皮细胞癌7例,恶性黑色素瘤4例,小细胞癌1例,小细胞恶性淋巴瘤1例。均采用手术治疗,其中6例因肿瘤侵及眶内,行眶内容剜出术,其余病例皆手术切除肿瘤,于术中送冰冻切片控制切缘(Mohs法)以保证术中完全切除肿瘤组织。眼睑缺损一期手术修复。结果6例行眶内容剜出术患者术后遗留明显的眼眶畸形,须二期行眼眶赝复或眼眶畸形整复术。68例术后眼睑外观满意,视功能无影响。结论眼睑恶性肿瘤多发生于老年人,其中基底细胞癌居第一位,其次为睑板腺癌,鳞状上皮细胞癌占第三位。治疗为手术切除加一期缺损修复,睑板结膜瓣滑行修复眼睑缺损具有操作方便、术后眼睑外形满意的优点。     

A novel approach to the management of severe facial disfigurement in neurofibromatosis type 1

A 12-year-old girl with neurofibromatosis type 1 underwent subtotal anterior exenteration of a blind, unsightly, buphthalmic eye and orbital/eyelid plexiform neurofibroma. Simultaneously, a channeled Medpor orbital implant was positioned to reduce a meningoencephalocele resulting from severe sphenoid wing dysgenesis. Two-stage osseointegration was subsequently performed to permit secure fitting of an orbital prosthesis. This patient's cosmesis and social interaction were improved markedly with this surgical approach for disfiguring ocular, eyelid, and orbital disease due to neurofibromatosis type 1.     

Locally Advanced Sebaceous Cell Carcinoma (T3) of Eyelid: Incidence and Pattern of Nodal Metastases and Combined Modality Management Approach

Background: Sebaceous carcinoma (SbCC) is a rare malignancy that often mimics benign conditions. Lymphatic involvement, large T3 tumors herald a dismal survival for patients. We present our series of 13 cases of locally advanced SbCC of the eyelid treated at a surgical oncology unit and describe the clinical profile, patterns of nodal spread and recurrence pattern in this subset of SbCC.

Methods: A retrospective analysis of case records was carried out for patients presenting with orbital tumors between January 1997 and April 2010 in the department of Surgical Oncology, AIIMS, New Delhi, India. All patients underwent orbital exenteration and superficial parotidectomy with neck dissection was added to patients with clinically significant lymphadenopathy. All patients who underwent OE after 2002 were advised radiotherapy as adjuvant therapy. The end point was development of recurrence or end of two year follow up period which ever occurred earlier.

Results: Thirteen patients underwent orbital exenteration. Eleven patients had clinically palpable lymphadenopathy. Ten patients (76.9%) had pathologically confirmed metastatic nodes. Parotid lymph node involvement was present in all patients (100%); two of these ten patients also had level II b cervical lymph node involvement. Recurrence was observed in seven patients (53.8%). All recurrences were loco-regional only and no systemic metastases was seen. There were only two recurrences in the group that received PORT.

Conclusions: Eyelid SbCC is a loco-regionally aggressive malignancy and adequate disease control can be achieved with combined modality approach of radical surgery followed by post operative radiotherapy.     

Myxofibrosarcoma of the orbit: case report and review of literature

A 17-year-old man was referred with a 6-month history of painless left inferior eyelid edema. Ophthalmic examination was normal except for the presence of a reddish conjunctiva in the left eye medial canthus. MRI demonstrated a 20-mm well-defined extraconical mass between the medial and inferior rectus muscle without destruction of the medial orbital wall. A low-grade myxofibrosarcoma was diagnosed on orbital biopsy. The tumor was locally excised, but the patient categorically refused left exenteration or conformal radiotherapy. He accepted to have annual clinical and radiologic checkup. To date, 2 years later, he has been followed up with no evidence of tumor recurrence. Orbital localization of myxofibrosarcoma is extremely rare, with only 2 cases reported in the literature. The differential diagnosis could be difficult: here, a fibromyxoid sarcoma and a myxoid variant of dedifferentiated liposarcoma have been especially discussed in terms of their histopathologic features. Risk of metastasis is related to the tumor grade. Management of these tumors associates surgery with orbital exenteration or globe-sparing approaches and postsurgical adjuvant radiation therapy.     

Basal cell carcinoma of the eyelid associated with Gorlin-Goltz syndrome

PURPOSE: To describe the ophthalmic and systemic features in a series of patients initially seen with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Of 105 consecutive patients with eyelid basal cell carcinoma managed at an Ocular Oncology Center between January 1973 and December 1999, four patients with Gorlin-Goltz syndrome were identified. METHODS: The ophthalmic and systemic features, management, and outcome of patients with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome were analyzed. The published literature on Gorlin-Goltz syndrome, specifically related to genetics, systemic features, ophthalmic associations, and prophylactic management strategies, was reviewed. MAIN OUTCOME MEASURES: Response of the eyelid basal cell carcinoma to treatment and the final systemic condition were the main outcome measures. RESULTS: All four patients had a family history of Gorlin-Goltz syndrome. The systemic manifestations included multiple basal cell carcinomas in all the patients, frontal bossing or increased occipitofrontal circumference in three patients, palmar pits in two patients, odontogenic keratocyst in one patient, ectopic calcification in one patient, and bifid rib in one patient. The mean age at the detection of the first basal cell carcinoma was 30 years (range, 16-38 years). All four patients had multiple basal cell carcinomas on the face and elsewhere. The eyelid basal cell carcinoma was advanced with orbital infiltration in three patients, one of whom opted for palliative radiotherapy, whereas the other two underwent orbital exenteration. The fourth patient, who had localized recurrent basal cell carcinoma in the upper eyelid, was treated with excision and eyelid reconstruction. At the final follow-up (mean, 41 months), eyelid basal cell carcinoma was cured in three patients and stable in one patient. No patient had life-threatening sequelae of Gorlin-Goltz syndrome. CONCLUSIONS: Gorlin-Goltz syndrome is a rare autosomal dominant cancer predisposition syndrome that may be associated with eyelid basal cell carcinoma. The associated systemic findings may be a clue to the diagnosis of this condition. It is important to recognize Gorlin-Goltz syndrome when a patient has multiple basal cell carcinomas or when a young patient with eyelid basal cell carcinoma is seen by an ophthalmologist, because lifelong monitoring is essential for patient management.     

Orbital Exenteration: A 10-Year Experience of a General Oncology Hospital

Background: The aim of this study is to review the 10-year experience with orbital exenteration, determining the indications, clinical features of patients and the factors that cause the need for exenteration.

Methods: The records of patients that underwent exenteration between 1997 and 2007 were reviewed retrospectively. The age, sex, the location of tumour, the duration of symptoms, histopathological diagnosis, and previous recurrences were noted.

Results: Orbital exenteration was performed on 68 patients. Bone resection in 6 and sinusectomy in 13 patients was combined with exenteration. The major indication was secondary orbital tumours accounting for 95.6% of the series and the eyelid and conjunctival tumours were the most frequent. Seven different types of histopathology were encountered: squamous cell carcinoma (31 cases), basal cell carcinoma (26 cases), malignant melanoma (5 cases), sebaceous cell carcinoma (2 cases), rhabdomyosarcoma (2 cases), mucoepidermoid carcinoma (1 case) and adenoid cystic carcinoma (1 case). The rate of previous recurrence was 85.7% in eyelid tumours and 42.1% in the conjunctival tumours.

Conclusions: Large proportion of the patients that underwent exenteration had a history of previous unsuccessful treatment and a long duration of disease. Because exenteration of the orbit is a disfiguring procedure, the incidence must be reduced by early presentation, prompt treatment, and adequate observation to preserve the eye.     

The use of latex biomembrane in exenteration: case report

The latex biomembrane is a material from Hevea brasiliensis and has angiogenic properties, cellular adhesion and extracellular matrix formation. It has been used for the treatment of ulcers in the inferior extremities caused by diabetes, chronic vascular insufficiency and timpanic perforations. In this study we report a case of biomembrane use to promote granulation in the orbital cavity after exenteration in a male, 72 years, who came into our service with a left eyelid lesion for two years. The ocular examination revealed a hyperemic conjunctiva, corneal thinning, cataract and ocular perforation in the left eye. The right eye had no alterations. There was an expansive and ulcerated lesion with orbital septum, frontal bone and lamina papiracea invasion confirmed by tomography. A total exenteration of the cavity was made and the biomembrane was applied to the affected site. Changes were made every 24 hours in the first seven days, and every other day in the following seven days. The biomembrane had to be taken from the patient because no granulation could be seen. Just one occurred and a half month later. Exenteration is a procedure in which all of the orbital content is removed. If the eyelids are included it is called total exenteration. There are many exenteration techniques as spontaneous granulation, skin grafts, miocutaneous flap, etc. In this case we used a material which has been successfully applied for other wounds, but with no efficiency in our experience. More studies are necessary so that we can present benefits to our patients in the ophthalmologic area with the latex biomembrane.     

161例眼眶内容物摘除术的病因分析

目的探讨眼眶内容物摘除术的病因。方法回顾性分析1981年1月～2007年12月行眼眶内容物摘除术的161例（161眼）患者的病例资料,其中男94例,女67例;年龄2～82岁,分析其原因。结果161例眼眶内容物摘除术中,46例眼眶原发性肿瘤、110例眼眶继发性肿瘤和5例非肿瘤性病变。共有26种病理类型,主要包括视网膜母细胞瘤（RB）67例,恶性黑色素瘤20例,鳞状细胞癌12例,腺样囊性癌10例。按原发病变部位位于眼球者81例,眼眶者51例,结膜者15例,眼睑者11例,鼻咽及副鼻窦者3例。结论眼眶原发性恶性肿瘤和继发性恶性肿瘤是眼眶内容物摘除术的主要原因。     

Eyelid sebaceous gland carcinoma in a young Caucasian man

BACKGROUND: Eyelid sebaceous gland carcinoma is rarely observed in young Caucasian men. HISTORY AND SIGNS: A 28-year-old man was referred for a recurring chalazion of the eyelid that had been operated four times in the past two years. The chalazion-like lesion of the external third of the upper left eyelid was associated with a cystic lesion. Cytology of the fluid in the cyst and histology of the lesion were compatible with a sebaceous cell carcinoma. Magnetic resonance imaging revealed that the cystic lesion associated with the eyelid tumour was extending into the orbit. THERAPY AND OUTCOME: According to current clinical practice and experience, to increase the chance of survival of the patient, an orbital exenteration was conducted to remove the sebaceous cell carcinoma in total. CONCLUSIONS: Although rare, one should be aware that an eyelid sebaceous cell carcinoma can occur in a young Caucasian man and this diagnosis should be evoked in case of a recurrent chalazion.     

Multimodal therapy in the management of primary orbital mesenchymal chondrosarcoma

AIM:To evaluate the ophthalmic manifestations,radiographic features,and prognosis of Chinese patients with primary orbital mesenchymal chondrosarcoma(MCS).METHODS:The study included 6 cases with primary orbital MCS treated at Tianjin Eye Hospital from January 2009 to December 2019.Patitent ophthalmic manifestations,radiographic features,diagnosis,pathology,therapeutic regimens,and prognosis were retrospectively reviewed.RESULTS:Six patitents with primary orbital MCS were identified.The mean age at the first visit was 33y(range,25-42y).All six patients displayed manifestations of exophthalmos,diplopia,limitation of eye displacement,upper eyelid oedema,decreased visual acuity and ptosis.The mean disease history and range were 5 and 2-8mo,respectively.The tumors were located in the superonasal extraconal compartment(2/6,33.3%),intraconal compartment(2/6,33.3%),and bitemporal extraconal compartment(2/6,33.3%),respectively.Radiographic features were a well-defined,orbital mass with calcification and ossification on computed tomography(CT),and marked heterogenous enhancement on dynamic magnetic resonance imaging(MRI).Five patients were treated with tumor resection and one patient received orbital exenteration.Five patients in the cohort received postoperative radiation therapy,two patients received chemotherapy,and one patient did not receive postoperative adjuvant therapy because he refused.The histopathologic classification revealed a tumour composed of a mixture of mature chondroid tissue surrounded by small,round,and undifferentiated mesenchymal cells.Immunohistochemistry revealed Bcl-2,vimetin,CD99,and S-100 were expressed were expressed.After surgeries,two patients have developed a local recurrence.The median recurrence time of 58 mo(52-64 mo).One patient had distant recurrence included the lungs occurred 52mo after the initial surgery.CONCLUSION:The possibilty of orbital MCS need to be considered when a painless,slowly growing orbital mass with calcification and ossification.From our experience,trimodality treatment of radiation therapy,chemotherapy and surgery maybe the best option.Orbital MCS has a high tendency for late recurrence,regular long-term follow-up after complete excision is mandatory.     

Plaque radiotherapy for selected orbital malignancies: preliminary observations: the 2002 Montgomery Lecture,part 2

PURPOSE: To describe the principles and preliminary results of plaque brachytherapy for selected orbital malignancies. METHODS: A custom-designed Iodine-125 plaque, designed to deliver a target dose of 50 Gray, was placed surgically in the region from which an orbital malignancy was partially resected. The mean dose to the target area was 50 Gray. The initial and follow-up patient data were reviewed. RESULTS: Of the 8 patients, the diagnosis was adenoid cystic carcinoma of the lacrimal gland (4 cases), orbital invasion by basal cell carcinoma (2), orbital extension of conjunctival melanoma (1), and metastatic carcinoma (1). Of the 4 with adenoid cystic carcinoma, there was microscopic residual tumor after excision and orbital exenteration was considered. Three have tumor control with follow-up of 1, 3, and 6 years. One patient required exenteration for recurrence separate from the field of brachytherapy and is free of tumor after 10 years. All 4 patients are alive and well with tumor control. Of the 2 patients with orbital extension of basal cell carcinoma, tumor control without recurrence has been achieved in both after 2 years. The patient with orbital metastasis responded to plaque radiotherapy, with no orbital recurrence, but died of systemic metastasis. The patient with orbital melanoma had local orbital recurrence separate from the area of irradiation and is currently being treated for systemic metastasis. CONCLUSIONS: Based on preliminary observations, plaque radiotherapy appears to be a reasonable alternative to exenteration and external irradiation for selected orbital malignancies.