Adult Orbital Langerhans Cell Histiocytosis With Frontal Bone Involvement

Sokol, Jason A.; Kazim, Michael; Kelly, Kara M.; Lantos, George; Leung, Loh-Shan; Baron, Edward

doi:10.1097/iop.0b013e31819ac2d9

Cited by 2 publications

(4 citation statements)

References 6 publications

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“…Different treatment modalities are used to treat unifocal orbital disease. The prognosis of unifocal disease is excellent after surgical resection (1)(2)(3)(4).…”

Section: Discussionmentioning

confidence: 99%

A 22‐Year Old Woman with Right Eyelid Swelling

Vugt¹,

Kesari²,

Muller³

et al. 2016

Brain Pathology

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A 22-year old female presented with a five-week history of right retroorbital pain and eyelid swelling. She denied headaches, loss of vision, focal neurological symptoms or ocular symptoms. On exam she had tenderness over right eyebrow and temporal bone. Her past medical history was unremarkable. An ophthalmologist initially saw her and a CT-scan was ordered. The scan showed an orbital bony lesion, which contained a solid component as well as a fluid level without calcifications or a bony matrix ( Figure 1a). A subsequent MRI revealed a contrast-enhancing lesion eroding the roof of the orbit posterolaterally on the right (Figure 1b). Involvement of the inner table of the temporal bone was also demonstrated on MRI. The patient was referred to our center at this time for further evaluation. A CT angiogram was performed to assess the vascularity of the lesion and whether or not any preoperative embolization would be of utility. On the angiogram no vascular abnormalities could be identified intracranially. One month after her initial presentation she successfully underwent a bifrontal craniotomy with resection of the orbito-temporal lesion and placement of a prosthetic right orbital roof. GROSS AND MICROSCOPIC PATHOLOGYGross examination revealed non-descript brown-tan and hemorrhagic soft tissues adherent to fragments of partially eroded bone. A separate specimen submitted as "dural margin" was composed of brown, hemorrhagic, lobulated, and somewhat cystic soft tissue adherent to the outer surface of normal dura. Hematoxylin and eosin stained sections of the specimens showed nodular aggregates of large mononuclear cells (Figure 1c) with high nuclear-to-cytoplasmic ratios characterized by indented and clefted nuclei (Figure 1d). These cells contained amphophilic cytoplasm, had distinct cytoplasmic borders, and showed positivity for both S-100 and CD1a by immunohistochemistry (Figures 1e and 1f, respectively). These nodules of atypical cells were accompanied by an exuberant mixed inflammatory infiltrate composed of many lymphocytes, plasma cells, and eosinophils (Figure 1g). Occasional multinucleate giant cells were seen throughout the inflammatory infiltrate (Figure 1h). Scattered CD68-positive histiocytes (Figure 1i) that were negative for both S-100 and CD1a were seen adjacent to atypical cells within the lesional nodules and within the mixed inflammatory infiltrate. What is your diagnosis? Figure 1. Brain Pathology 26 (2016) 425-426

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“…Different treatment modalities are used to treat unifocal orbital disease. The prognosis of unifocal disease is excellent after surgical resection (1)(2)(3)(4).…”

Section: Discussionmentioning

confidence: 99%

A 22‐Year Old Woman with Right Eyelid Swelling

Vugt¹,

Kesari²,

Muller³

et al. 2016

Brain Pathology

View full text Add to dashboard Cite

show abstract

“…5,7 As with typical LCH, orbital LCH has been described at length in children, 3,5,6,[8][9][10][11] but to our knowledge, fewer than 20 cases have been reported in adults. 7,10,12 Classically, orbital LCH will present with unilateral orbital pain or headache with proptosis. Localized redness and swelling, as in our case, may also be present.…”

Section: Primary Langerhans Cell Histiocytosis Of the Lacrimal Gland mentioning

confidence: 99%

“…15 Some ophthalmologists advocate localized therapy with surgical excision with or without intralesional steroids. 3,5 In contrast, oncologists identify orbital LCH as a central nervous system (CNS) risk for issues such as diabetes insipidus (DI) 12,15 and mandate 6 months of chemotherapy. With their proximity to the CNS, these lesions cannot simply be treated conservatively.…”

Section: Primary Langerhans Cell Histiocytosis Of the Lacrimal Gland mentioning

confidence: 99%

“…5 Patients with extended orbital and periocular involvement may be more likely to experience development of CNS disease, such as DI. 5,12 Of the multiple chemotherapeutic options available to treat multisystem LCH, results are varied, 17 thus making the prognosis more guarded in these patients. Macular atrophy in a case of abetalipoproteinemia as only ocular clinical feature Abetalipoproteinemia (Bassen-Kornzweig syndrome) is a rare, autosomal recessive, multisystem disorder caused by an absence of apolipoprotein-B.…”

Section: Primary Langerhans Cell Histiocytosis Of the Lacrimal Gland mentioning

confidence: 99%

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