11 July 2022: Textbook Updates

We have posted updates of the following topics:

CNS & pituitary tumors > Pineal tumors > Desmoplastic myxoid tumor of the pineal region, SMARCB1 mutant
by Mariana Voudouri, M.D., George Zanazzi, M.D., Ph.D.
Topic summary: Pineal region neoplasm with ovoid or spindle shaped cells, containing inactivating alterations of the SMARCB1 / INI1 locus and variable amounts of desmoplasia and myxoid matrix. Small to medium sized cells with ovoid to spindle shaped nuclei. M:F = 2:3; median patient age: 36.6 years old. Prognostic factors are not yet defined but reported prognosis much better than atypical teratoid / rhabdoid tumor. Surgical resection is the treatment of choice; chemotherapy or radiotherapy has been given to a subset of patients.

Nasal cavity, paranasal sinuses, nasopharynx > Other malignant tumors > Sinonasal glomangiopericytoma
by Jinping Lai, M.D., Ph.D.
Topic summary: Uncommon soft tissue tumor of perivascular myoid differentiation with hemangiopericytoma-like vasculature. CTNNB1 mutation with beta catenin oncogenic activation (beta catenin nuclear staining on IHC). Nasal obstruction, mass, polyps, difficulty breathing. Broad age range (5 – 90 years); mean in seventh decade; F > M (1.2:1). Excellent prognosis after surgery (5 year survival ~90%). Surgery (polypectomy or wide surgical resection) is the treatment of choice; radiation could be used for nonsurgical candidates.

Prostate gland & seminal vesicles > Inflammation > Prostatitis
by Kenneth A. Iczkowski, M.D.
Topic summary: Prostatitis is a clinical term only, so it should not be diagnosed on pathology reports. Either lymphocytes, neutrophils, eosinophils or histiocytes. Chronic prostatitis / chronic pelvic pain syndrome (CPPS), also known as NIH category III prostatitis, abacterial prostatitis or prostatodynia, is the most common urological diagnosis in men older than 50 years. Prognostic factors: depends on the timely diagnosis and treatment and type of prostatitis. Treatment: difficult because antibiotics penetrate poorly into prostate.

Skin nontumor > Panniculitis > Lipodermatosclerosis
by Syeda Qasim, M.D., Shyam x, M.D.
Topic summary: Lipodermatosclerosis presents as a chronic fibrosing disease commonly associated with venous insufficiency, arterial ischemia or thrombophlebitis. Lipodermatosclerosis is also known as venous stasis panniculitis or hypodermitis sclerodermaformis. Common, middle aged or older people. Histologically shows an increase in dermal fibroblast proliferation and procollagen gene expression, suggesting involvement of these factors. Treatment: lifestyle – weight reduction, physical activity. Medical treatment: stanozolol, pentoxifylline, hydroxychloroquine.

Transfusion medicine > Transfusion reactions & complications > Transfusion associated graft versus host disease
by Deyze Badarane, M.D., M.P.H., C.P.H., Kyle Annen, D.O.
Topic summary: Transfusion associated graft versus host disease (TA-GVHD) is a rare, fatal, delayed reaction to blood transfusion. Blood products, such as whole blood, red blood cells, platelets, granulocytes and plasma that have not been frozen (e.g., liquid plasma), can contain viable T lymphocytes and cause TA-GVHD. Clinical manifestations affect the skin, GI tract, liver and bone marrow; severe neutropenia leading to untreatable infection is the primary cause of death in TA-GVHD. Prevention: gamma irradiation of cellular blood products, pathogen reduction technology.

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