Sclerosing polycystic adenosis

Sclerosing polycystic adenosis (SPA) is a rare benign neoplasm that primarily affects the salivary glands. It was first described in 1996 by Smith et al. and is characterized by its resemblance to fibrocystic disease of the breast and sclerosing adenosis.

Epidemiology

SPA is a rare condition, with fewer than 100 cases reported in the literature. It can occur at any age but is most commonly diagnosed in middle-aged adults. There is no known gender predilection.

Pathology

SPA is characterized by a polycystic architecture, with cysts lined by a double layer of epithelial cells. The stroma is typically sclerotic, and there is often a lymphocytic infiltrate. The lesion can be unifocal or multifocal, and it can involve any of the major or minor salivary glands. The most commonly affected site is the parotid gland.

Clinical Presentation

Patients with SPA typically present with a slow-growing, painless mass. Some patients may experience pain or discomfort. On examination, the mass is usually firm and non-tender. SPA can be associated with Sjögren's syndrome and other autoimmune diseases.

Diagnosis

The diagnosis of SPA is typically made on histopathological examination of a biopsy specimen. Imaging studies such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) can be helpful in determining the extent of the disease and planning treatment.

Treatment

The treatment of choice for SPA is surgical excision. In some cases, where the disease is extensive or surgery is not feasible, radiotherapy may be considered.

Prognosis

The prognosis for patients with SPA is generally good. The disease is benign and does not metastasize. However, recurrence after surgical excision is common.

See Also

• Salivary gland neoplasm
• Sjögren's syndrome
• Fibrocystic disease