Angiolymphoid hyperplasia with eosinophilia

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Article initiated by:

Abdulrahman Alfarhan, MD

All contributors:

Michael T Yen, MD, Shannon S. Joseph, M.D., Abdulrahman Alfarhan, MD

Assigned editor:

Shannon S. Joseph, M.D.

Review:

Assigned status Up to Date

 by Shannon S. Joseph, M.D. on December 2, 2024.

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Disease

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign neoplastic lesion. It is characterized by vascular proliferation and secondary inflammatory reaction. Wells et al. were the first to describe the disease entity as persistent subcutaneous nodule and massive eosinophilic infiltration. It is unclear whether ALHE shows gender-specific trends, and previous reports in the literature lack general consensus. ALHE typically presents as solitary or multiple chronic dermal nodules occurring primarily in the head and neck region. Extracutaneous involvement, such as orbital adnexa, has been also reported in the literature . ALHE lesion is typically non-tender and non-pulsatile and can present as fullness of the eyelid with mechanical ptosis, proptosis, and lacrimal gland enlargement. A closely related lesion known as Kimura’s disease (KD) shares some similarities with ALHE, and it was once thought a spectrum of the same disease. Some reports may be seen pointing to a close relationship between the two. Published literature has shown conflicting response to the role of imaging in ALHE due to the nonspecific radiological features ALHE can present with. Therefore, the final diagnosis relies on histopathological features of this entity.

General Pathology

Histopathologic examinations of angiolymphoid hyperplasia with eosinophilia that reveals fibrovascular tissue infiltrated with chronic inflammatory cells and abundance of plasma cells. Also, hyperplasia of the small arterioles and venules surrounded by numerous eosinophils

Light microscopic studies can accurately define the diagnosis. Histologic patterns include vascular endothelial proliferation with a variety of inflammatory cell infiltrate such as lymphocytes and eosinophils lining the vascular endothelial beds . The tumor often arises in the lumen of the capillaries, arterioles, and venules or in the wall and expands out into the surrounding soft tissue . Immunostaining for vascular markers can highlight the obscurity of vessels lumen. The endothelial cells lining the vascular lumina are classically thick, plumb, and epithelial-like. They also can look like a nest of epithelial cells rather than vessels with lumen. The vascular endothelium was frequently found to demonstrate cytoplasmic vacuoles and nuclear atypia. However, this specific histological feature is not pathognomonic for ALHE. Proliferating vessels tend to be arranged in lobules with a brisk of lymphocytic background and germinal center formations. In addition, background eosinophils are usually abundant and robust. Yet, some cases may have sparse inflammation and a few scattered eosinophils are present.

Signs and Symptoms

Clinical photographs for patients with orbital angiolymphoid hyperplasia with eosinophilia shows an anterior orbital mass involving the temporal side with swelling, fullness, and ptosis. Photo courtesy of Dr. Abdulrahman Alfarhan.

ALHE should be suspected in patients with lid swelling, proptosis, ptosis, diplopia, dystopia, and/or ocular dysmotility. Pain is not typically present in patients with ALHE, and this should raise the suspension of an underlying malignancy. The duration of symptoms until diagnosis can vary from few weeks to 12 years , and the mean duration of ALHE symptoms reported in the literature is 18 months. In 75% of all reported cases of orbital ALHE, patients were more than 30 years of age, but age at diagnosis can be as early as 4 years and as late as 86 years. ALHE is generally unilateral but can present as bilateral.

Imaging

The magnetic resonance imaging shows extraocular high signal lesion on T2

The choice of imaging study depends on the clinical context. CT scan and MRI can help localize and define the extent of the lesion in reference to 3-dimensional anatomy. However, when iodinated contrast media cannot be performed due to patient’s allergy to iodinated contrast, MRI is recommended for exact lesion characterization. ALHE can involve the orbit, lacrimal gland, eyelids, or less commonly conjunctiva. The most common site of involvement was superotemporal orbit. CT scan shows intense enhancement of the lesion following intravenous injection of contrast media. If the lacrimal gland is to be involved, inflammatory changes are usually noted. Lacrimal gland involvement is associated with preseptal thickening and infiltration of the superolateral compartment of the extraconal fat with enlargement of the extraocular muscles. The lesion is typically well circumscribed and located in the extraconal orbital compartment, abutting the lateral orbital wall and lateral zygoma. MRI can show the lacrimal gland exhibiting high T2/FLAIR and low T1 signal intensity with homogeneous enhancement on the post-contrast images. Evidence of globe infiltration was not reported in the literature.

Differential diagnosis

The differential diagnosis of ALHE primarily includes soft tissue masses of the orbit, including but not limited to:

• Kimura's disease (or eosinophilic lymphogranuloma)
• Orbital hemangioma
• Lymphangioma
• Orbital pseudotumor
• Hodgkin’s lymphoma
• Castleman’s disease
• Langerhans cell histiocytosis
• Angioimmunoblastic T cell lymphoma
• Eosinophilic granulomatosis with polyangiitis
• Drug-induced and parasitic lymphadenitis
• Thyroid Eye Disease (Graves' disease)

Management

Treatment is primarily intended to alleviate symptoms and to address aesthetic concerns. Similar to many tumors involving the orbit, surgical excision or debulking is the preferred treatment modality and is often curative. Other forms of treatment, including intralesional steroid, oral propranolol, interferon therapy, and irradiation, have all been reported. Observation awaiting spontaneous regression can be considered in areas where extensive surgical excision can be disfiguring. Recurrences can take place in bilateral disease or after incomplete excision.