We have posted updates of the following topics:
Breast > Ductal carcinoma in situ > Paget disease
by Jasmine Vickery, M.D., Anna Biernacka, M.D., Ph.D.
Mammary Paget disease (MPD) is a proliferation of malignant glandular epithelial cells (in situ carcinoma) in the nipple areolar epidermis. Cutaneous manifestation is due to tumor cells involving the epidermis and disrupting intercellular junctions. Relatively uncommon; occurs in 1 – 4% of women and 1 – 2% of men with breast cancer. In symptomatic cases, the diagnosis is primarily clinical and confirmed by histologic examination. Prognosis largely depends on the presence, extent and characteristics of the underlying tumor. Extent of surgery is determined by the extent of underlying breast carcinoma. Patients with limited disease are candidates for breast conserving therapy with central lumpectomy and whole breast irradiation.
Bone marrow neoplastic > Bone marrow – plasma cell and lymphoid neoplasms > Plasma cell neoplasms > MGUS-IgM
by Shuchi Zinzuwadia, B.S., Shweta S. Zinzuwadia, B.S., Maryam F. Raouf, M.D., Rita Gupta, M.D., Ameet R. Kini, M.D., Ph.D.
IgM monoclonal gammopathy of undetermined significance (MGUS) is characterized by < 3 g/dL of monoclonal IgM protein, < 10% clonal. Bone marrow clonal lymphoplasmacytic cells or clonal plasma cells < 10% of cellular elements. Prevalence of MGUS is 2 – 3% of adults over 50 years. Diagnosis of IgM MGUS is based on the presence of a serum IgM monoclonal protein < 3 g/dL, fewer than 10% clonal lymphoplasmacytic or plasma cells in the bone marrow and lack of evidence of progression to lymphoplasmacytic lymphoma / Waldenström macroglobulinemia, IgM multiple myeloma or AL amyloidosis. Risk factors for progression are initial concentration of the monoclonal protein (≥ 1.5 g/dL) and abnormal serum free light chain ratio. Treatment is not recommended for asymptomatic patients with IgM MGU; “Wait and watch” approach is usually followed.
Bone & joints > Notochordal lesions > Chordoma
by Jesse Hart, D.O.
Malignant tumor with notochordal differentiation. Typically involves the clivus, sacrococcygeal bones or vertebrae. Most commonly arises in the 40 – 60 year old age group (may occur at any age). Most cases are sporadic but rare cases may be associated with benign notochordal tumor. Diagnosis is based on morphology, immunohistochemical findings and anatomic location of the tumor. Median survival is 7 years; 5 year overall and disease free survival are 61% and 71%; 10 year overall and disease free survival are 41% and 57%. Treatment: usually surgery followed by radiation or radiation alone in poor surgical candidates; poor response to chemotherapy.
CNS nontumor > Toxic and metabolic disorders > Hepatic encephalopathy
by Palgun Nisarga, M.D., Kymberly A. Gyure, M.D.
Spectrum of neurocognitive abnormalities occurring in patients with acute or chronic liver disease or portosystemic shunting. Elevated blood ammonia levels considered to play central role. Epidemiology: chronic alcoholism, acute drug poisoning, status post portosystemic bypass, hepatic failure patients. Acute hepatic encephalopathy: cerebral edema and increased intracranial pressure (cause of death). Prognosis based on percent of viable hepatic parenchyma in acute hepatic failure. Treatment of precipitating event; reduce blood. ammonia concentration using nonabsorbable disaccharides or antibiotics
Soft tissue > Uncertain differentiation > Synovial sarcoma
by Farres Obeidin, M.D., Borislav A. Alexiev, M.D.
Malignant soft tissue tumor of uncertain histogenesis with variable epithelial differentiation. Can occur in or around any tissue in the body. Accounts for 5 – 10% of all soft tissue sarcomas; median age: 35. Etiology: despite the name, the cells of origin are not synovial cells; histogenesis is still debated. Biopsy is required for definitive diagnosis. Radiotherapy and adjuvant chemotherapy have been evaluated in high risk situations (tumors > 5 cm or difficult to resect).