Primary orbital melanoma arising in an atypical diffuse (plaque-like) blue naevus/melanocytosis: a case report and review of literature

Background Primary orbital melanoma is a rare disease and can occasionally develop from a pre-existing neoplasm of the blue naevus family of melanocytic lesions. Case presentation Herein we report a rare case of primary orbital melanoma arising from an unusual atypical diffuse (plaque-like) blue naevus/melanocytosis. A 27 year old man presented with mild pain and swelling of the left eye. Magnetic Resonance Imaging revealed a left lateral episcleral orbital mass and an incisional biopsy confirmed the diagnosis of malignant melanoma. Skin-sparing total left orbital exenteration was performed. Histopathological examination of the exenteration specimen revealed a primary orbital melanoma arising in a pre-existing blue naevus like melanocytosis. We demonstrate the evidence for histological progression, characterise the molecular profile of this tumour and discuss the related literature. Conclusions This case emphasises the importance of a meticulous clinicopathological correlation in recognising such a tumour as a primary orbital melanoma rather than a metastasis, which is managed differently.

Orbital myiasis on recurrent undifferentiated carcinoma in the COVID-19 era: a case report and brief review of the literature

Background Myiasis is defined as the infestation of living tissues by Diptera larvae. Ophthalmic involvement occurs in less than 5% of cases. As the most uncommon type of involvement, orbital myiasis usually affects patients with poor personal hygiene, a low socioeconomic status, a history of surgery, and cancer. Findings In January 2020, an 89-year-old man presented to the Oculoplastic Department of Farabi Eye Hospital (Iran) with a history of left-side progressive orbital mass for six months. A large infiltrative mass of the left orbit with extension to the globe, periorbita, and adnexa was remarkable at the presentation, and its appearance suggested malignancy. Our findings persuaded us to perform exenteration and histopathological evaluation which were reported as “undifferentiated carcinoma”. Regular follow-up visits were recommended. In June 2020, with a 3-month delay, the patient presented with the recurrence of the mass complicated with mobile alive larva. Examinations revealed numerous maggots crawling out of an ulcerative and foul-smelling lesion. He stated that fear of COVID-19 infection postponed his follow-up visit. The patient underwent immediate mechanical removal of larvae, followed by wide local excision of the mass. Conclusion Patients with carcinoma of the adnexal tissues seem to be more prone to myiasis infestation even though it is an uncommon disease. Since COVID-19 is an ongoing pandemic with no end in sight appropriate protocols should be implemented to prevent loss of follow-up in these high risk patients.

Rare Orbital Metastasis Originating from Ampullary Adenocarcinoma

Background: Orbital metastasis from ampullary carcinoma is rare, with no previously reported cases. Case presentation: We report the case of a 60-year-old man who complained of a right-sided headache, blurred vision, progressive proptosis, ptosis, and right eye pain for 3 months. His past medical history included an ampullary adenocarcinoma stage IIIA treated via the Whipple procedure and adjuvant chemoradiotherapy 1 year ago. However, he was lost to follow-up. Computed tomography of the orbit showed a soft tissue lesion in the right orbital fossa measuring 3.3 × 2 × 2 cm. An orbital mass biopsy demonstrated an intestinal-type adenocarcinoma that tested positive for cytokeratins 7 and 20 and CDX2 on immunohistochemical staining. The pathologic diagnosis was metastatic adenocarcinoma from the ampulla of Vater. Despite oncological treatment, the patient’s illness progressed. He received palliative treatment and died 1 month later. Conclusions: We presented a rare case of orbital metastasis from ampullary adenocarcinoma. This should be considered in the differential diagnosis of patients with a history of ampullary adenocarcinoma who present with symptoms referring to the relevant locations.

3D MHD simulation of a pulsationally-driven MRI decretion disc

We explore the pulsationally driven orbital mass ejection mechanism for Be star disc formation using isothermal, 3D magnetohydrodynamic (MHD) and hydrodynamic simulations. Non-radial pulsations are added to a star rotating at 95 per cent of critical as an inner boundary condition that feeds gas into the domain. In MHD, the initial magnetic field within the star is weak. The hydrodynamics simulation has limited angular momentum transport, resulting in repeating cycles of mass accumulation into a rotationally-supported disc at small radii followed by fall-back on to the star. The MHD simulation, conversely, has efficient (Maxwell αM ∼ 0.04) angular momentum transport provided by both of turbulent and coherent magnetic fields; a slowly decreting midplane driven by the magnetorotational instability and a supersonic wind on the surface of the disc driven by global magnetic torques. The angle and time-averaged properties near the midplane agree reasonably well with a 1D viscous decretion disc model with a modified $\tilde{\alpha }=0.5$, in which the gas transitions from a subsonic thin disc to a supersonic spherical wind at the critical point. 1D models, however, cannot capture the multi-phase decretion/angular structure seen in our simulations. Our results demonstrate that, at least under certain conditions, non-radial pulsations on the surface of a rapidly rotating, weakly magnetized star can drive a Keplerian disc with the basic properties of the viscous decretion disc paradigm, albeit coupled to a laminar wind away from the midplane. Future modeling of Be star discs should consider the possible existence of such a surface wind.

Orbital myeloid sarcoma misdiagnosed for subperiostal hematoma: a case report

Background Myeloid sarcoma is a solid tumor that consists of immature myeloid cells occurring at an extramedullary site. It can present before, concurrent with, or after the diagnosis of acute myeloid leukemia or other myeloproliferative diseases, and a proportion of patients never develop bone marrow infiltration. Only a few isolated cases of pediatric orbital myeloid sarcoma have been reported, and they are often associated with a high misdiagnosis rate. Case report We report a rare case of pediatric orbital myeloid sarcoma associated with blunt trauma in a 3-year-old Caucasian male patient, which was clinically and radiologically misdiagnosed for orbital subperiostal hematoma. The patient underwent a surgical intervention to drain the hematoma when an orbital mass was found. The microscopic, immunologic, and genetic features of the tumor and the myelogram were in favor of LAM2, and the patient was started with chemotherapy with a favorable evolution within 18 months follow-up. Conclusion Orbital myeloid sarcoma usually exhibits clinical and radiological features that can be easily misleading, especially if it happens de novo or as the first manifestation of acute myeloid leukemia. Only a few isolated cases have reported and proposed trauma as a trigger event of the onset of this type of tumor proliferation, but further investigations and evidence are needed to support this hypothesis.

Malignancies in Immunoglobulin G4-Related Ophthalmic Disease

Purpose: Clinical phenotypes in Immunoglobulin G4-related disease (IgG4-RD) according to the affected organs affected have different risks of malignancies. We attempt to determine the association of malignances with IgG4-related ophthalmic disease (IgG4-ROD). Design: Retrospective cohort study. Methods: Review of medical records, orbital images and histopathology reports in a territory-wide cohort of patients fulfilling the “probable” or “definite” comprehensive diagnostic criteria of IgG4-RD from 2005-2019. Findings: Among 122 patients who had biopsies taken from adnexal lesions including lacrimal glands (n=108), orbital mass (n=30), infiltrated orbital fat (n=10), conjunctiva (n=2) or extraocular muscles (n=3), 16 (13%) developed malignancies over 73±48months’ follow-up. There were 9 cases of ocular adnexal lymphoma (OAL) and 7 extra-orbital malignancies. Compared with the general population, the incidence of OAL was significantly higher (standardized incidence ratios, SIRs=10.0, 95%CI=4.5-17.6) while that of extraorbital malignancy was similar. The SIR was highest within the first year (SIR=46.7, 95%CI=18.5-87.6) when 7 OAL were concomitantly diagnosed. Patients who developed OAL or extra-orbital malignancies were older than other patients when diagnosed of IgG4-ROD (64.9±7.1, 68.3±8.5 versus 55.2±15.0 years, p<0.05). Asymmetric lacrimal gland enlargement (78% versus 13%), lack of frontal (0% versus 12%) or infraorbital nerve enlargement (0% versus 36%)were associated with OAL (all p<0.05). Pre-treatment serum IgG4 level or pattern of extraorbital involvement was similar among patients with or without malignancies. Conclusion: In this biopsy-proven IgG4-ROD cohort, 7% developed OAL which was 10 times higher than the general population. Patients with asymmetric lacrimal gland enlargement or without trigeminal nerves involvement radiologically were associated with OAL.

Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency

We report a case of IgG4-RD in a patient with high IgG4 levels, low functional antibodies, and low IgM levels. He presented with bilateral orbital pseudotumors and, after initial improvement on corticosteroids, relapsed with recurrent pleural effusion and pelvic pseudotumor. He had a grossly elevated serum IgG (1905 mg/dl) with elevations in all IgG subclasses but marked elevation in IgG4 (412 mg/dl), low IgM, and low pneumococcal antibodies. Orbital mass biopsy showed polyclonal lymphocytic infiltration and increased IgG4 plasma cells. The patient was started on prednisone and tried several immunosuppressive medications including mycophenolate mofetil, methotrexate, hydroxychloroquine, and azathioprine with decrease in size of the orbital pseudotumor. During a period when the patient stopped his medications, the pseudotumor enlarged with new development of recurrent pleural effusions. He was also found to have a pelvic mass that was biopsy positive for IgG4 proliferation. This case with progression to multiorgan involvement highlights the importance of identifying patients with IgG4-related disease. In contrast to previous cases with normal-to-high IgM, the IgM was low with impaired functional antibodies.

Phakomatous Choristoma of the Orbit with Inferior Oblique Muscle Involvement

Purpose: We report a case of phakomatous choristoma presenting as an orbital tumor with involvement of the inferior oblique muscle. Case summary: A 2-month-old male infant presented to our clinic with a right orbital mass that had been present since birth. Magnetic resonance imaging demonstrated a homogenous enhanced well-defined mass located in the inferomedial portion of the right orbit without bone erosion. By transconjunctival orbitotomy, the orbital tumor invading the inferior oblique muscle was identified and resected. Histopathology showed a thick basement membrane surrounding pseudoglandular structures embedded in a collagenous stroma, psammomatous calcific foci in the stroma, and eosinophilic material in the lumen. Immunohistochemical analysis showed positive staining for S-100 and cytokeratin. On histopathological evaluation, the tumor was diagnosed as phakomatous choristoma. Conclusions: To our knowledge, this is the first report in South Korea of phakomatous choristoma of the orbit with involvement of the inferior oblique muscle. Although rare, phakomatous choristoma should be included in the differential diagnosis of tumors occurring on the inferomedial side of the orbit.