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• W2079796375 abstract "A58-year-old woman presented to our institution with a 2-year history of bilateral swelling of the parotid glands associated with dry eyes and dry mouth. Raynaud phenomenon had been diagnosed 3 years previously, at which time she had classic episodes of digital triphasic color changes (white, blue, then red) from cold exposure associated with digital ulcers. Two years before the current evaluation, Sjögren syndrome was diagnosed because of the parotid swelling and persistent xerostomia and sicca symptoms. Although autoimmune markers were negative at that time, the patient was treated empirically with hydroxychloroquine sulfate. Therapeutic doses of hydroxychloroquine (200 mg twice daily) for 2 years failed to improve her symptoms. At presentation to our institution, she had no frank arthritic, neurologic, cutaneous, renal, gastrointestinal, or pulmonary involvement due to Sjögren syndrome. She denied fever, chills, night sweats, anorexia, and weight loss. Her medical history was notable only for chronic hypertension. She was a previous smoker but reported no alcohol use, intravenous drug use, or high-risk sexual behavior. Her family history was unremarkable. Physical examination revealed a well-appearing woman with normal vital signs except for an elevated blood pressure level of 156/78 mm Hg. Both parotid glands were markedly enlarged from the angle of the mandible to the inferior angle of the ear. The oral cavity was extremely dry, and the submandibular glands were firm. Examination of the skin revealed only a small ulcer on the tip of the right third finger. Palpable adenopathy was not present. The remainder of the physical examination findings were unremarkable. Laboratory test results were as follows (reference ranges shown parenthetically): hemoglobin, 11.9 g/dL (12.0-15.5 g/dL); leukocytes, 5.5 × 109/L (3.5-10.5 × 109/L); neutrophils, 4.49 × 109/L (1.7-7.0 × 109/L); monocytes, 0.2 × 109/L (0.3-0.9 × 109/L); lymphocytes, 0.65 × 109/L (0.9-2.9 × 109/L); platelet count, 169 × 109/L (150-450 × 109/L); lactate dehydrogenase, 210 U/L (104-236 U/L); erythrocyte sedimentation rate, 14 mm/h (0-29 mm/h); creatinine, 1.4 mg/dL (0.7-1.2 mg/dL); aspartate aminotransferase, 36 U/L (12-31 U/L); alanine aminotransferase, 39 U/L (929 U/L); rheumatoid factor, 450 IU/mL (<15 IU/mL); antinuclear antibody (ANA), 8.0 (<1.0); anti–doublestranded DNA antibody, 9 IU/mL (<25 IU/mL); anticentromere antibody, positive; anti–SS-A antibody, negative; anti–SS-B antibody, negative; and serum cryoglobulins, 4% (negative). 1.Which one of the following is the most likely diagnosis in this patient? a.CREST syndromeb.Systemic lupus erythematosus (SLE) with associated Raynaud phenomenonc.Hepatitis Cd.Sjögren syndrome with associated Raynaud phenomenone.Cryoglobulinemia CREST syndrome consists of a constellation of signs and symptoms: calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Approximately 70% to 90% of patients with this syndrome have anticentromere antibodies. Although our patient had this autoimmune marker, she had only one of the features of the CREST syndrome (Raynaud phenomenon). Thus, despite positive antibody test results, our patient does not have CREST syndrome. Systemic lupus erythematosus with associated Raynaud phenomenon is also an unlikely diagnosis in our patient who had an elevated ANA level but negative results of testing for anti–double-stranded DNA antibodies, which are more specific than ANA for SLE. Furthermore, she had no clinical symptoms of SLE, such as the classic malar rash or typical arthritic pain. Hepatitis C is an important diagnosis to consider in a patient with slightly elevated levels of aspartate aminotransferase and alanine aminotransferase, suggestive of mild hepatitis. However, our patient had no history of high-risk sexual behavior or evidence of chronic liver disease, such as jaundice, spider angiomas, palmar erythema, or asterixis. Hepatitis serologic testing should be undertaken, but infection with hepatitis C does not explain the patient's current symptoms. Sjögren syndrome with associated Raynaud phenomenon is the most likely diagnosis in this patient. The classic bilateral parotid enlargement and history of xerostomia and keratoconjunctivitis (sicca symptoms) are diagnostic despite the absence of specific antibodies for Sjögren syndrome. Anti–SS-A antibodies are detected in 90% of patients with primary Sjögren syndrome and anti–SS-B antibodies in 60%.1Tan EM Robinson CA Nakamura RM ANAs in systemic rheumatic disease: diagnostic significance.Postgrad Med. September 1985; 78 (145-148.): 141-142PubMed Google Scholar Associated Raynaud phenomenon can be diagnosed on the basis of the patient's digital ulcer and history of classic digital changes. Cryoglobulinemia is associated with several different disorders, such as chronic infections (most commonly hepatitis C), autoimmune disorders, and lymphomas. Affected patients typically present with nonsystemic small-vessel vasculitis, palpable purpura, urticaria, and cutaneous ulcerations; peripheral neuropathy, arthalgia, and arthritis are also common. Our patient had low titers of cryoglobulins but none of the other classic symptoms. Therefore, cryoglobulinemia is not the most fitting diagnosis at this time. The patient was advised to continue taking hydroxychloroquine. She returned several months later for evaluation of a new firm nodule in the left parotid gland. Examination revealed persistent generalized bilateral parotid enlargement and a well-circumscribed 5 × 6-cm firm, fixed, nontender, nonerythematous mass in the left parotid gland. No lymph nodes were palpable. 2.Which one of the following is the most likely cause of the new parotid mass? a.Acute mumpsb.MALT (mucosa-associated lymphoid tissue) lymphomac.Warthin tumor or papillary cystadenomad.Acute suppurative parotitise.Sarcoidosis Mumps, a parotitis caused by a paramyxovirus, is an acute contagious disease primarily affecting children younger than 15 years.2Mandel L Surattanont F Bilateral parotid swelling: a review.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002; 93: 221-237Abstract Full Text Full Text PDF PubMed Scopus (100) Google Scholar A viral prodrome usually precedes the onset of painful unilateral or bilateral parotid swelling. Our patient's age and her painless parotid swelling preclude a diagnosis of mumps. MALT lymphoma occurs in 20% of persons with Sjögren syndrome and chronic parotid enlargement.3Kassan SS Thomas TL Moutsopoulos HM et al.Increased risk of lymphoma in sicca syndrome.Ann Intern Med. 1978; 89: 888-892Crossref PubMed Scopus (893) Google Scholar The chronic inflammation in Sjögren syndrome causes a lymphocytic infiltration of the parotid gland, which eventually can lead to the proliferation of a monoclonal B-cell lymphoma. Therefore, MALT lymphoma is the likely diagnosis in this patient. Warthin tumor is a benign epithelial tumor originating from salivary gland tissue, either unilaterally or bilaterally.2Mandel L Surattanont F Bilateral parotid swelling: a review.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002; 93: 221-237Abstract Full Text Full Text PDF PubMed Scopus (100) Google Scholar It is strongly associated with smoking, and the mass is generally soft, freely movable, and painless, features inconsistent with our patient's condition. Acute suppurative parotitis is usually caused by Staphylococcus aureus infection in severely dehydrated patients with markedly decreased salivary flow. It occurs typically in newborns, systemically ill or immunocompromised patients, and those recovering from abdominal surgery who have inadequate fluid replacement. Our patient had none of these predisposing conditions, and no signs of infection were detected on physical examination. Parotid enlargement occurs in 10% to 30% of patients with sarcoidosis,2Mandel L Surattanont F Bilateral parotid swelling: a review.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002; 93: 221-237Abstract Full Text Full Text PDF PubMed Scopus (100) Google Scholar an important and often overlooked disease to consider in the differential diagnosis of bilateral parotid swelling. Parotid sarcoidosis is generally diagnosed on the basis of a classic clinical history and consistent pulmonary radiological findings. Our patient's clinical history was not consistent with sarcoidosis. To further evaluate the bilateral parotid gland enlargement and the left parotid mass, computed tomography (CT) of the head and neck was performed. The CT scan revealed a hypoenhancing, well-circumscribed mass in the anterolateral |aspect of the superficial lobe of the left parotid gland measuring approximately 2 × 2.2 × 2 cm. No lymphadenopathy was detected. 3.Which one of the following is the best next step in evaluating the mass and confirming the diagnosis? a.Fine-needle aspiration (FNA) biopsyb.Open incisional biopsyc.Magnetic resonance imagingd.Total parotidectomye.Superficial parotidectomy The role of FNA in evaluating parotid masses requires insight into the likely diagnosis. In patients in whom a MALT lymphoma is suspected, the FNA biopsy specimen usually does not provide sufficient material for cytologic study and immunophenotyping. However, for most other parotid malignancies, FNA biopsy can aid diagnosis and surgical planning. Because we suspected a MALT lymphoma in our patient, FNA biopsy was considered inappropriate. Open incisional biopsy is contraindicated because of the high likelihood of “tracking” potential head and neck tumor cells.4Stewart A Blenkinsopp PT Henry K Bilateral parotid MALT lymphoma and Sjögren's syndrome.Br J Oral Maxillofac Surg. 1994; 32: 318-322Abstract Full Text PDF PubMed Scopus (38) Google Scholar Tracking does not generally occur with lymphoma tumor cells, but in our patient the histology of the tumor was unknown, and open incisional biopsy could not be recommended. Magnetic resonance imaging has not been shown to provide superior radiological information for evaluating suspected MALT lymphomas and cannot confirm the diagnosis. Computed tomography remains the imaging modality of choice. Although total parotidectomy would yield a diagnosis, it is not generally recommended for diagnostic purposes, especially in cases with superficial lobe involvement. Furthermore, a total parotidectomy may leave the patient with facial nerve paralysis if the nerve has to be sacrificed during surgery. Superficial parotidectomy is the best next step in our patient because enough tissue can be removed to establish the diagnosis, and most, if not all, of the tumor can be resected. Given our high clinical suspicion for a MALT lymphoma in the left parotid gland, a left superficial parotidectomy with removal of 2 periparotid lymph nodes was performed. Pathological examination revealed an extranodal marginal zone B-cell lymphoma of MALT type. All lymph nodes were negative for disease. 4.What, if anything, should be done next to stage this patient's lymphoma to guide therapy? a.CT of the chest, abdomen, and pelvisb.Bone marrow biopsyc.Dissection of nodes in the neckd.Upper endoscopye.No further evaluation needed Computed tomography of the chest, abdomen, and pelvis is the most effective and useful method for staging tumors before initiation of therapy. A CT scan accurately reveals distant metastases that could alter treatment decisions. A bone marrow biopsy is not recommended for staging purposes because MALT lymphoma is rarely seen in the bone marrow. Node dissection has not proved beneficial for staging purposes. Surgeons routinely remove some of the surrounding lymph nodes when performing the superficial parotidectomy, yielding sufficient tissue to document local spread of disease. Thus, further neck dissection is unnecessary. Upper endoscopy is used only when gastric MALT lymphoma is suspected because of the well-known association with Helicobacter pylori infection. Upper endoscopy is not recommended for staging other MALT lymphomas such as those seen in the salivary glands. Further evaluation with CT is the recommended standard of practice for staging MALT lymphoma. Therefore, “no further evaluation needed” is inappropriate in this situation. Computed tomography of the chest, abdomen, and pelvis revealed no evidence of lymphadenopathy or distant metastases. Because our patient had parotid involvement only, the lymphoma was designated stage 1AE: 1 = 1 site of disease, A = no B symptoms, and E = presence of extranodal disease. 5.Which one of the following would be the most appropriate treatment for our patient at this time? a.Chemotherapy appropriate for non-Hodgkin lymphomab.Local radiotherapyc.Antibiotics for H pylori infectiond.Total parotidectomye.Observation only Chemotherapy is an option for patients with MALT lymphoma who have evidence of distant metastases. The chemotherapeutic regimens are similar to those used in patients with classic B-cell non-Hodgkin lymphoma, such as CHOP (cyclophosphamide, hydroxydaunomycin [doxorubicin], Oncovin [vincristine], and prednisone). Because our patient had no evidence of lymphadenopathy or metastases, chemotherapy was not indicated. Local radiotherapy after superficial parotidectomy has proved beneficial in a few case studies of patients with local disease only and is the most appropriate treatment for our patient. A low dose (0.40 Gy) of radiation is usually administered, and some investigators have advocated bilateral irradiation to prevent the development of MALT lymphoma in the unaffected parotid gland.5Balm AJ Delaere P Hilgers FJ Somers R Van Heerde P Primary lymphoma of mucosa associated lymphoid tissue (MALT) in the parotid gland.Clin Otolaryngol. 1993; 18: 528-532Crossref PubMed Scopus (27) Google Scholar, 6Suchy BH Wolf SR Bilateral mucosa-associated lymphoid tissue lymphoma of the parotid gland.Arch Otolaryngol Head Neck Surg. 2000; 126: 224-226Crossref PubMed Scopus (21) Google Scholar Antibiotic treatment is used for patients with gastric MALT lymphoma because of its well-known association with H pylori infection but is not standard treatment for parotid MALT lymphomas. A total parotidectomy is indicated when the deep lobe is involved with either primary parotid malignancies or benign parotid tumors. It would not be appropriate for our patient, whose MALT lymphoma involved only the superficial lobe. Although some investigators recommend “observation only” after surgery for local disease, the current literature suggests that local radiotherapy after superficial parotidectomy produces the best outcome. Several months later, the patient underwent evaluation for a right superficial parotidectomy to establish possible involvement of MALT lymphoma in that gland. During this evaluation, cutaneous and renal vasculitis developed, thought to be associated with Sjögren syndrome. The patient is still recovering from these complications, and further surgery and radiotherapy have been postponed until recovery is complete. MALT lymphomas were first described by Isaacson and Wright7Isaacson P Wright DH Malignant lymphoma of mucosa-associated lymphoid tissue: a distinctive type of B-cell lymphoma.Cancer. 1983; 52: 1410-1416Crossref PubMed Scopus (1440) Google Scholar in 1983. Because they are found in mucosa-associated lymphoid tissue and not in lymph nodes, they are classified as extranodal non-Hodgkin lymphomas. Most MALT lymphomas are of B-cell rather than T-cell lineage. They occur in the gastrointestinal tract, salivary and thyroid glands, lungs, and occasionally other organs. The gastrointestinal tract is the primary source of MALT, predominantly the stomach. In the gastrointestinal tract, MALT works as part of the immune system's protective mechanism against luminal foreign bodies. MALT lymphomas are also found in organs that are devoid of MALT, such as the salivary glands, thyroid gland, and lungs; these lymphomas appear to have an affinity for the extranodal glandular epithelium found in these organs.8Isaacson PG Spencer J Malignant lymphoma of mucosa-associated lymphoid tissue.Histopathology. 1987; 11: 445-462Crossref PubMed Scopus (700) Google Scholar Often, organs in which MALT lymphomas occur are involved by a chronic inflammatory process, such as Sjögren syndrome in the salivary glands, Hashimoto thyroiditis in the thyroid gland, and, possibly, interstitial pneumonia in the lungs, that elicits an immune response and lymphocytic infiltration of the tissues. Chronic inflammation of the parotid gland, such as seen in Sjögren syndrome, causes periductal lymphocytic infiltration to process luminal antigens. Normal parenchyma is replaced, and ductal epithelial cells proliferate to form lymphoepithelial islands causing parotid enlargement. About 4% to 7% of patients with benign lymphoepithelial proliferation will develop a monoclonal B-cell clone8Isaacson PG Spencer J Malignant lymphoma of mucosa-associated lymphoid tissue.Histopathology. 1987; 11: 445-462Crossref PubMed Scopus (700) Google Scholar acquired from MALT normally present in the gastrointestinal tract. With time, this clonal population of cells will proliferate into a MALT lymphoma. The antigenic stimulus causing proliferation of a monoclonal B-cell clone remains speculative, except in the well-known case of H pylori and Helicobacter heilmanni infections associated with gastric MALT lymphomas. Clinically, MALT lymphomas of the parotid gland are most frequently observed in patients with Sjögren syndrome or those with human immunodeficiency virus infection due to immunosuppression. Patients often present with painless, diffuse bilateral parotid enlargement and typically have xerostomia or sicca symptoms if Sjögren syndrome is present. Diagnosis is often difficult, requiring CT imaging for initial evaluation. Fine-needle aspiration biopsy is not recommended because it is nondiagnostic in most cases. Superficial parotidectomy is the recommended diagnostic procedure. In patients with only superficial lobe involvement, complete resection can be accomplished concurrently with superficial parotidectomy. Clinicians should be aware that MALT lymphomas (like other low-grade lymphomas) can transform into a more aggressive process, usually diffuse large cell lymphoma, that is associated with a worse prognosis and is more difficult to treat. Transformation should be suspected when a patient has symptoms of rapid tumor growth, B symptoms, or an elevated lactate dehydrogenase level. If transformation or recurrence is suspected, the patient should undergo repeated biopsy and appropriate therapy based on the histological findings. With or without treatment, most MALT lymphomas have minimal associated morbidity and mortality.2Mandel L Surattanont F Bilateral parotid swelling: a review.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002; 93: 221-237Abstract Full Text Full Text PDF PubMed Scopus (100) Google Scholar The tumor is indolent and remains localized for long periods but can spread locally to lymph nodes and distantly to other organs and/or nodes. Computed tomography is the best tool for staging MALT lymphoma. Therapeutic options vary depending on the extent of the disease. If the lymphoma is localized, treatment with superficial parotidectomy and local radiotherapy is recommended. If disseminated disease is present, standard chemotherapy for non-Hodgkin lymphoma should be instituted. Of note, the primary antigenic stimulus for gastric MALT lymphomas seems to be H pylori infection. Eradication of this bacteria has resulted in tumor remission in 50% to 70% of patients.9Bayerdorffer E Neubauer A Rudolph B MALT Lymphoma Study Group et al.Regression of primary gastric lymphoma of mucosa-associated lymphoid tissue type after cure of Helicobacter pylori infection.Lancet. 1995; 345: 1591-1594Abstract Full Text PDF PubMed Scopus (912) Google Scholar For salivary, thyroid, and lung MALT lymphomas, the antigenic stimulus remains elusive. If a stimulus is identified, further treatment options can be explored. CorrectionMayo Clinic ProceedingsVol. 79Issue 2PreviewIncorrect dose: In the Residents’ Clinic article by Jones and Swaroop entitled “58-Year-Old Woman With Dry Mouth and Swollen Parotid Glands,” published in the September 2003 issue of the Mayo Clinic Proceedings (Mayo Clin Proc. 2003;78:1145–1148), an incorrect dose (0.40 Gy) was printed in the ninth line of the right-hand column on page 1147. The sentence should read, “A low dose (25–30 Gy) of radiation is usually administered, and some investigators have advocated bilateral irradiation to prevent the development of MALT lymphoma in the unaffected parotid gland. Full-Text PDF" @default.
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