Specific types include:
— Bone. Primary lymphoma may affect any bone but usually the long bones are involved. If the skull is involved, central nervous system invasion may occur.
— Breast. Primary lymphoma of the female breast mimics carcinoma. Staging finds lymph node, marrow, or other extranodal sites of involvement in half the cases.
— Central nervous system. Involvement of the leptomeninges may produce headache, stiff neck, and cranial nerve impairment; brain involvement can result in headache, lethargy, papilledema, focal neurologic signs, or seizures; spinal cord involvement can result in back pain, extremity weakness, paresis, and paralysis. Usually aggressive type of diffuse large B-cell lymphoma. Intracerebral lymphoma is a feature of acquired immunodeficiency syndrome.
— Chest and lung. Primary pulmonary lymphoma may present as a solitary mass in the lung and require lung biopsy for diagnosis. Primary chest wall lymphoma can be accompanied by fever, sweating, and dyspnea and require excisional biopsy for diagnosis. Primary endobronchial lymphoma may follow lung transplantation and cause airway obstruction.
— Endocrine glands. Primary adrenal lymphoma usually presents bilaterally and may lead to adrenal insufficiency. Primary thyroid lymphoma often develops on the background of autoimmune (Hashimoto) thyroiditis. Primary pituitary lymphoma can result in pituitary insufficiency, including diabetes insipidus.
— Eye. Ophthalmic lymphoma is the most common orbital malignancy and includes lymphoma involving the eyelids, conjunctiva, lacrimal sac, lacrimal gland, orbit, or intraocular space. Ophthalmic lymphoma accounts for approximately 7 percent of primary extranodal lymphoma cases.
— Gastrointestinal tract. This is the most common form of primary extranodal lymphoma, accounting for approximately one-third of cases. The most common site of involvement is the stomach, followed by the ileum, cecum, colon, and rectum. The liver, pancreas, and gallbladder may also be the site of extranodal lymphoma. Symptoms are related to the site involved (e.g., nausea, vomiting, diarrhea, bleeding).
— Genitourinary tract. Primary lymphoma of the testes presents as painless enlargement and may be bilateral. Primary lymphoma of the ovary is often bilateral and presents as abdominal masses sometimes felt on abdominal or pelvic examination. Cases limited to the uterus, uterine cervix, vagina, or vulva may occur. Lymphomatous involvement of both kidneys usually presents with renal failure. Bilateral ureteral involvement presents with obstructive renal failure. Primary lymphoma of the bladder or of the prostate may occur.
— Heart. Primary cardiac lymphoma may involve the heart or pericardium. Patients may present with dyspnea, edema, arrhythmia, or pericardial effusion with tamponade. Masses may occur in the right atrium (most common), pericardium, right ventricle, left atrium, or left ventricle.
— Paranasal sinuses. May present with local pain, upper airway obstruction, rhinorrhea, facial swelling, or epistaxis. Usually diffuse large B-cell lymphoma in the United States and Western Europe and T-cell and NK cell lymphoma in Asia.
— Skin. The three main types of cutaneous B-cell lymphoma are: primary cutaneous marginal zone B-cell lymphoma, primary cutaneous follicular center B-cell lymphoma, and primary cutaneous large B-cell lymphoma (leg type). The first two are indolent lymphomas and the last type is an aggressive lymphoma. These lymphomas may present as soft tissue masses, mimicking sarcoma, until a biopsy and histopathologic diagnosis is obtained.
— Spleen. Primary splenic lymphoma is rare since concomitant marrow involvement is present in most cases. The issue of whether splenic lymphoma is extranodal arises but since lymphoma is usually confined to the red pulp and not the white pulp, it can be considered extranodal.