Giulio Bonavolontà

AN ANALYSIS OF THIRTY CASES OF ORBITAL LYMPHANGIOMA : PATHOPHYSIOLOGIC CONSIDERATIONS AND MANAGEMENT RECOMMENDATIONS

Thirty cases of orbital lymphangioma were reviewed. Clinical, imaging, and microscopic findings were integrated to develop a pathophysiologic construct and management guidelines. The basic lesion might be considered an abortive vascular system which arborizes among normal structures. Intrinsic hemorrhage expands portions of the small-caliber network into large blood cysts, prompting clinical recognition. While major hemorrhage led to early surgery in 12 cases, long pretreatment intervals could be analyzed in 17 others. Twelve patients had second hemorrhages of varied magnitude, from 2 weeks to 15 years after initial recognition; five patients did not in an average of 6.8 years. Some blood cysts contracted spontaneously. Among 23 operated cases, 12 patients had major new bleeds from 4 days to 12 years after initial surgery; 11 patients did not in an average of 4.2 years. Poor final visual acuity was associated with multiple surgeries. The authors advocate conservatism in surgical case selection and in operative dissection.

Epithelial lacrimal gland tumors: pathologic classification and current understanding.

OBJECTIVE To apply the updated epithelial salivary gland classification scheme to a large cohort of lacrimal gland tumors so as to provide an updated lacrimal gland tumor classification scheme. METHODS A retrospective multicenter cohort study of 118 cases of epithelial neoplasia was undertaken. Main outcome measures included pathologic analysis, subtyping, and survival. RESULTS Of 118 cases, 17 (14%) were reclassified using the proposed expanded classification scheme based on the current World Health Organization classification of salivary gland tumors. The most frequent neoplasms were pleomorphic adenoma and adenoid cystic carcinoma, of which we highlight more unusual histologic features. Three tumors were found to be unclassifiable with the updated scheme, with 2 having histologically malignant features. Deficiencies and variations in pathologic assessment were noted. Variation in the histologic findings of pleomorphic adenoma and assessment of the extent of invasion of carcinoma ex pleomorphic adenoma were highlighted. CONCLUSIONS The use of the more histologically diverse classification of salivary gland tumors can be successfully applied to the epithelial lacrimal gland neoplasms. This expanded classification system led to reclassifying 14% of cases. Currently, there are no consistent pathologic standards for processing and evaluating these lesions.

Fine needle aspiration (FNA) biopsy of orbital masses: a critical review of 51 cases

Fine needle aspiration (FNA) biopsy of orbital masses: a critical review of 51 cases

An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011.

Purpose: To evaluate the frequencies of orbital space-occupying lesions seen at single orbital unit in a period of 35 years. Methods: In this retrospective case series, the authors reviewed the medical records of 2,480 consecutive patients referred to the authors’ Orbital Unit for evaluation of an orbital mass between 1976 and 2011. The final diagnosis in each case was established by a combination of history, ocular findings, diagnostic imaging, and histopathologic analysis, when available. The number and percentage of benign and malignant tumors were determinated, also according to the age of the patients and the tumor location within the orbit. This study adhered to the principles of the Declaration of Helsinki. Results: Of the 2,480 lesions, 1,697 (68%) were benign and 783 (32%) were malignant. The most frequent benign tumors were dermoid cyst (14%) and cavernous hemangioma (9%). The most common malignant tumors were non-Hodgkin lymphoma (12%), basal cell carcinoma (3%), and orbital metastases (3%). In patients younger than 60 years, benign tumors are more frequent, whereas in patients older than 60 years, the frequency of malignant tumors increased. Regarding the distribution in the orbit, the most common tumors were dermoid cyst (206 cases) in the upper-outer quadrant, mucocele (155 cases) in the upper-inner quadrant, basal cell epithelioma (35 cases) in the lower-inner quadrant, cavernous hemangioma (68 cases) in the lower-outer quadrant, and meningioma (90 cases) in central space. Most of the tumors were located in the upper-outer quadrant. In the lower-inner quadrant, malignant tumors were more frequent than benign tumors. Conclusions: The authors’ findings highlight the distinctive nature of the orbital oncology experience at an ocular oncology department.

Orbital venous-lymphatic malformations (lymphangiomas) mimicking cavernous hemangiomas

PURPOSE To illustrate that orbital venous-lymphatic malformations (lymphangiomas) may rarely simulate cavernous hemangiomas. METHODS Retrospective case review. RESULTS Five patients were identified from a series of 85 patients with venous-lymphatic malformations. The age range was 21 to 69 years, and all cases presented with a history of slowly progressive or long-standing proptosis. Computerized tomography revealed relatively homogeneous intraconal masses that were well defined anteriorly. Two of the cases had expansion of the orbit, and one had focal calcification. The three who had magnetic resonance imaging showed heterogeneous contrast enhancement. The preoperative diagnosis in every case was cavernous hemangioma, and intraoperatively the lesions resembled cavernous hemangiomas. However, posterior dissection was difficult in all patients because of dense adhesions and, in one case, led to a central retinal artery occlusion. The histology was characteristic of orbital venous-lymphatic malformations in all five cases. CONCLUSIONS Deep orbital venous-lymphatic malformations presenting in adulthood may be rarely confused with cavernous hemangiomas. In doubtful cases, significant intralesional heterogeneity, best seen on magnetic resonance imaging, and focal calcification may help distinguish the two entities. This differentiation is important, because dissection of venous-lymphatic malformations is fraught with more complications than surgical excision of a cavernous hemangioma.

Primary Optic Nerve Sheath Meningioma in Children

Primary optic nerve sheath meningioma represents a proliferation of meningothelial cap cells of the arachnoid villi within the optic nerve sheath. Patients younger than 20 years of age make up less than 5% of all cases of pediatric primary optic nerve meningiomas. Histopathologically, the most common subtypes in children are transitional (54%) and meningotheliomatous (38%). This tumor has been called aggressive in the pediatric population, with surgical excision recommended. However, the tumor may spread intraorbitally, intracranially, or intraocularly after subtotal surgical resection. Recent studies examined the use of fractionated, stereotactic radiation in children with this tumor; however, follow-up is limited. Neurofibromatosis type 2 is concomitantly diagnosed in 28% of patients with pediatric primary optic nerve sheath meningioma. There have been no known deaths attributed primarily to this tumor.

Acinic cell carcinoma arising in a lacrimal gland. First case report

Acinic cell carcinoma is a rare neoplasm nearly always observed in the salivary glands. Our experience documents a case of this pathology, occurring in the lacrimal gland, never reported in the literature before. A 59‐year‐old white woman was admitted with reduced vision and a painless swelling in the area of the right lacrimal gland. Computerized axial tomography showed an intraorbital radio‐opaque mass compressing the eyeball from behind. The patient underwent right temporal orbitotomy and total removal of the lesion. The surgical specimen was constituted by a cystic mass containing citrine liquid. Microscopic examination proved the lesion to be an acinic cell carcinoma. Differential diagnostic problems and similarity to salivary gland acinic tumors are discussed.

Radiation therapy in primary orbital lymphoma: a single institution retrospective analysis

BackgroundPrimary orbital lymphoma is a rare disease that accounts for 10% of all orbital tumors. Radiotherapy on the orbital cavity is the treatment of choice for this unusual presentation of localized non-Hodgkins lymphoma (NHL). The aim of this study is to retrospectively evaluate the effectiveness and the toxicity of radiation treatment in patients with primary orbital lymphoma.MethodsForty-seven consecutive patients having primary orbital lymphoma treated in our department between May 1983 and September 2006 were investigated in a retrospective study. Either 60Co γ rays or 6 MV X rays were used to deliver daily fractions of 1.8 or 2.0 Gy, 5 times/week, with total doses ranging from 34.2 to 50 Gy. Forty-three patients had stage IE, three had stage II and one stage IV disease. Thirty-eight patients had marginal zone B-cell lymphoma, 5 diffuse large B cell lymphoma, 3 mantle cell lymphoma and 1 Burkitt lymphoma. Local control (LC), disease free survival (DFS), overall survival (OS) and late side effects were evaluated in all patients.ResultsWith a median follow up of 45 months, LC was obtained in 100% of patients. The estimated 5- and 7-year DFS rates were 75.8% and 55.3%, and the 5- and 7-year OS rates were 88.7% and 79.9% respectively. Acute toxicity was minimal. Late toxicity such as cataract, keratitis, retinopathy and xerophthalmia occurred respectively in 12 (25.5%), 5 (10.6%), 1 (2.1%), and 9 (19.1%) patients.ConclusionRadiotherapy is an effective and at the same time well tolerated treatment for primary orbital lymphoma.

Radiation late effects in children treated for orbital rhabdomyosarcoma.

BACKGROUND AND PURPOSE The experience resulting from large cooperative studies shows that correct radiation therapy at doses adequate to the tumor bulk are crucial for local control of rhabdomyosarcoma. The aim of the present study was to document the correlation between modalities and doses of radiotherapy and radiation side effects. PATIENTS AND METHODS Between 1980 and 1997, 19 patients affected by primary orbital rhabdomyosarcoma have been followed at the University Federico II of Naples. All but three patients, who received 45, 54 and 55 Gy respectively, have been treated by immediate radiation at the dose of 60 Gy, delivered in 2 Gy fractions, five times per week, by cobalt 60 megavoltage equipment. Combined chemotherapy using vincristine and vincristine plus dactinomycin on alternate weeks was also administered as part of induction therapy. RESULTS An overall survival rate of 94.7% was registered. In our patients the majority of radiation late effects were paid by orbit and ocular adnexa. Side effects to lens and ocular structures were fewer and of low grade. CONCLUSIONS Radiation therapy is still essential for local control of orbital rhabdomyosarcoma, however radiation side effects have to be carefully considered together with the therapeutic goal to be obtained.

Primary Ewing's sarcoma of the maxilla, a rare and curable localization: report of two new cases, successfully treated by radiotherapy and systemic chemotherapy

Primary maxillary localization of Ewings sarcoma is unusual. Involvement of facial bones is characterized by clinical and radiological features distinct from those commonly observed in other sites. Because of the above peculiarities a delay in diagnosis and thus in starting treatment is very probable in such cases. We report here two new cases of Ewings sarcoma localized to facial bones, successfully treated by local high dosage radiotherapy and systemic chemotherapy. Our experience suggests that, especially for particular sites not suitable to radical surgery, radiation therapy can represent an effective tool to achieve local control of the tumor.