Periocular apocrine adenocarcinoma presenting as an orbital mass: clinicopathological features and management in four patients

Purpose: Apocrine adenocarcinoma is a cutaneous adnexal malignancy which can rarely arise from ocular structures. In this retrospective study, we report our experience with four patients who had primary periocular apocrine adenocarcinoma initially presenting with an orbital tumor. Methods: Data extracted included demographics, clinical, imaging, and histopathological features, and treatment outcomes. Results: The definitive diagnosis was established after an incisional biopsy in all cases. Two patients were then managed with exenteration. The third patient underwent local resection followed by radiotherapy but had to be exenterated because of new tumor formation 7 years later. The fourth patient had to be managed with oral bicalutamide which kept the tumor stable for 3 years. Recurrence-free survival for the radical surgical treatment group was 10, 6, and 7 years respectively. Conclusion: Periocular apocrine adenocarcinoma may insidiously develop as an orbital mass without any clinically detectable primary eyelid skin or conjunctival lesions. This tumor must be in the differential diagnosis of medially located ill-defined orbital masses in patients over 50 years of age. Orbital exenteration appeared as an effective treatment of apocrine adenocarcinoma with orbital extension. Anti-androgenic treatment in an androgen receptor-positive tumor provided temporary local tumor control.

Role of Quantitative Diffusion-Weighted Imaging in Differentiating Benign and Malignant Orbital Masses

Aim To determine the role of diffusion-weighted imaging (DWI) with apparent diffusion coefficient (ADC) values in differentiating benign and malignant orbital masses. Materials and Methods After obtaining institutional ethical board approval and informed consent from all patients, an observational study was done for a period of 24 months in the radiology department of a tertiary care hospital in South India. Conventional magnetic resonance imaging and DWI using a 3T scanner was done for all patients with suspected orbital mass lesion. ADC value and clinicohistopathological correlation were studied for every patient. Chi-square test was used to compare the signal characteristics of DWI and ADC maps between benign and malignant lesions. A comparison of mean ADC values for benign and malignant masses was performed using Student’s t-test for independent samples. The cut-off value for ADC was obtained using the receiver operating characteristic (ROC) curve. Results Of 44 patients with orbital lesions, 70% were benign and 30% were malignant. There was a significant difference in the mean ADC values of benign and malignant orbital masses. Using ROC curve analysis, an optimal ADC threshold of 1.26 × 10−3 mm2/s was calculated for the prediction of malignancy with 100% sensitivity, 80.65% specificity, and 86.36% accuracy (95% confidence interval: 0.872, 1.00, p < 0.0001). Two ADC thresholds were used to characterize the orbital masses with more than 90% confidence. Conclusion Quantitative assessment of ADC is a useful noninvasive diagnostic tool for differentiating benign and malignant orbital masses. Malignant orbital lesions demonstrate significantly lower ADC values as compared with benign lesions.

Lacrimal ductal cyst of the medial orbit: a case report

Background The lacrimal ductal cyst (dacryops) is an uncommon clinical entity. It occurs anywhere that lacrimal gland tissue is present but most often appears as an expanding mass in the region of the lacrimal gland. The presence involving the medial part of the orbit is rare, ectopic location can be misleading in the differential diagnosis of orbital masses. The authors report a 53-year-old man who presented with dacryops occurred in an unusual location with significant clinical presentations. Case presentation A 53-year-old man had a painless mass located in the right superomedial orbit accompanied with foreign body sensation and lachrymation for two months, which had rapidly grown within 10 days. Decrease of visual acuity, high intraocular pressure (IOP) and limitation of extraocular movements in the right eye were present. The result of visual evoked potential (VEP) test suggested the impaired function of the optic nerve. Magnetic resonance imaging (MRI) studies revealed the presence of an isolated cystic lesion. The mass was completely removed via a transcutaneous approach, histopathologic findings were consistent with the lacrimal ductal cyst. The ocular motility and high IOP returned to normal. There had been no post-operative complications or signs of recurrence over five months follow-up. Conclusion Lacrimal ductal cysts can present in the medial orbit, clinicians should include this entity in the differential diagnosis of orbital masses and be aware of its variable presentations such as high IOP in this case. We comment on the fact that many reported cases of ectopic dacryops may be an extension of normal lacrimal gland tissue.

Filler Migration to the Orbit

Background Dermal filler injections continue to grow in popularity as a method of facial rejuvenation. This increase in the number of injections performed has resulted in an increasing number of types of filler-related complications. Objectives We report a series of cases where dermal filler injected in the face migrated to the orbit. Treatment methods and possible mechanisms of this newly reported complication are discussed. Methods A retrospective, multicenter analysis was performed on patients with dermal filler migration to the orbit after facial filler injections. Results Seven patients (6 females, 1 male; age range, 42-67 years) presented with orbital symptoms after filler injection and were subsequently found to have dermal filler in the orbit. Four out of 7 patients underwent orbitotomy surgery, 1 patient underwent lacrimal surgery, 1 patient had strabismus surgery, and 1 patient was treated with hyalurodinase injections. All patients have remained stable postoperatively. Conclusions Orbital complications secondary to migrated filler may occur long after the initial procedure. Because the site of the complication is distant from the injection site, patients and physicians may not immediately make the connection. Furthermore, this may lead to unnecessary examinations and a delay in diagnosis while looking for standard orbital masses. Dermal fillers should therefore be considered in the differential diagnosis of patients presenting with new-onset orbital masses. Level of Evidence: 4

Invasive Carcinoma Ex-Pleomorphic Adenoma of the Lacrimal Gland with a Cystadenocarcinoma Component: A Case Report and Review of the Literature

Lacrimal gland neoplasms comprise up to 18% of all orbital masses clinically and histologically. Much of our current core knowledge regarding lacrimal gland tumors stems from prior study of their more common counterparts, the salivary glands. The prognosis for each lacrimal gland tumor is contingent upon proper clinical evaluation and ultimately the histopathologic diagnosis. We describe a case of an invasive carcinoma ex-pleomorphic adenoma (Ca-ex-PA) with a cystadenocarcinoma component arising from the lacrimal gland in the absence of any previously diagnosed pleomorphic adenoma (benign mixed tumor) or prior incisional surgery. This case illustrates the importance of the histopathologic assessment including immunohistochemistry and genetic testing to narrow a differential diagnosis and potentially aid or guide therapy in the future. Our finding suggests that carcinoma of the lacrimal gland may be derived from previously undiagnosed and perhaps even subclinical pleomorphic adenoma.

FRI0210 ORBITAL PSEUDOTUMOR AMONG PATIENTS WITH GRANULOMATOSIS WITH POLYANGIITIS – DATA FROM THE POLISH REGISTRY POLVAS

Background:Orbital inflammatory masses have been described as the common manifestation of granulomatosis with polyangiitis (GPA) occuring in 7- 45% of patients.Objectives:Identification and characterization of patients with orbital pseudotumor among Polish patients based on the national vasculitis registry, POLVAS.Methods:Clinical presentation and management of all GPA patients fulfilling ACR criteria or Chapel Hill Consensus Conference definition included to the Polish registry POLVAS who developed orbital masses in the course of GPA were evaluated.Results:Ocular involvement was found in 114 (27%) of 417 GPA patients registered in POLVAS, 34 (8%) developed orbital masses. Mean patients’ age was 47.8 (range from 19-75) yrs., 23 (67%) were women. Forty four per cent of the patients developed tumor at the beginning of the disease, 56% during relapse. Patients’ characteristics on diagnosis of orbital mass: 24 cANCA, 2 pANCA, and 8 ANCA negative, 9% active smokers and 31% past smokers, 29% had localized disease, 21% early systemic and 50% systemic with organ involvement, 29% had other type of ophthalmological involvement before pseudotumor occurred, 88% had active paranasal sinus involvement, 41% lungs, 15% CNS, 15% skin and 6% heart manifestations. Thirty seven per cent of patients had positive nasal swabs cultures, 50% of which were positive for Staphylococcus aureus. In 65%, tumor occurred during steroid therapy (46% had prednisone more than 5mg/d) and 45% on immunosuppressive treatment (19% when treated with AZA, 16% MTX, 6,5% MMF and 3,5% CYC). Due to orbital mass 86.5% were treated with CYC and 13.5% with RTX. Twenty one per cent had complete remission of the pseudotumor, 76% partial remission and in 3% patients there was no response to the treatment; 43% developed visual impairment, 20% suffered from blindness.Conclusion:Orbital inflammatory mass was not common manifestation of GPA among our patients. The mass developed at the beginning or in the course of the disease, even during immunosuppressive treatment. Orbital masses have been resistant to therapeutic interventions and were accompanied by high risk of visual impairment.Disclosure of Interests:Anna Masiak: None declared, Marcin Ziętkiewicz: None declared, Krzysztof Wójcik: None declared, Katarzyna Wawrzycka-Adamczyk: None declared, Radoslaw Jeleniewicz: None declared, Marta Madej: None declared, Joanna Kur-Zalewska: None declared, Katarzyna Jakuszko: None declared, Małgorzata Wisłowska: None declared, Hanna Storoniak: None declared, Michał Komorniczak: None declared, Barbara Bułło-Piontecka: None declared, Iwona Brzosko: None declared, Małgorzata Stasiek: None declared, Eugeniusz Kucharz: None declared, Alicja Dębska-Ślizień: None declared, Maria Majdan Consultant of: Roche, Amgen, Speakers bureau: Roche, Amgen, Jacek Musiał: None declared, Zbigniew Zdrojewski: None declared

T2 mapping in orbital masses: preliminary study on differential diagnostic ability of T2 relaxation time

Background T2 mapping has been proven to be useful in tumor characterization. As to orbital masses, its diagnostic value needs to be investigated. Purpose To evaluate the usefulness of T2 mapping in orbital masses and the ability of T2 relaxation time in differentiating malignant from benign orbital masses. Material and Methods Forty-seven patients with solid orbital masses (33 benign and 14 malignant) who underwent T2 mapping examination for preoperative assessment were enrolled in the current study. T2 mapping was acquired using 16 TE values (range 12–192 ms; delta TE 12 ms). Mean T2 relaxation time was calculated based on the whole mass region of interest and compared between the malignant and benign groups using the unpaired t-test. Receiver operating characteristic curve analysis was adopted to calculate its diagnostic value. Results Malignant orbital masses showed significantly lower T2 relaxation time than benign masses (76.4 ± 13.0 ms vs. 119.1 ± 20.4 ms; P < 0.001). If setting a T2 relaxation time of 89.5 ms as the threshold value, optimal differentiating performance could be achieved (area under the curve 0.936; sensitivity 100.0%; specificity 87.9%; accuracy 91.5%; positive predictive value 77.8%; negative predictive value 100%). Conclusion T2 mapping and its derived T2 relaxation time could provide quantitative information and serve as a supplementary imaging marker for differentiating malignant from benign orbital masses.