Questions and Answers
What is Trichoepithelioma characterized by?
Islands of basaloid cells surrounded by an onion-skin pattern of fibrocytes.
What type of tumor is characterized by multiple circumscribed fibrotic nodules?
Epithelioid hemangioendothelioma.
What is the most likely diagnosis of a tumor showing tubular and ductal structures with hyaline stroma?
Pleomorphic adenoma.
What is the most likely diagnosis for a tumor with extensive cribriform architecture?
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What condition is described by persistent hyperinsulinemic hypoglycemia?
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Which histopathologic finding is NOT characterized by necrotic keratinocytes?
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From what are prostatic ductal adenocarcinomas derived?
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In infantile precursor B-lymphoblastic leukemia, what gene is most commonly rearranged?
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What infection is associated with severe acute villitis in the placenta?
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What is the most common chromosomal abnormality associated with clear cell renal cell carcinoma?
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What is the beta-catenin profile of bladder adenocarcinoma?
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What type of injury is typical for a lesion seen during a motor vehicle collision?
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What is the main cause of death in long-term survivors of cardiac transplantation?
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What is the correct diagnosis for a girl with a well-defined radiolucency in the distal femur?
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What is the diagnosis of a nodule with a characteristic pattern of medium-sized glands and hyalinized stroma?
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What is the hormone profile and translocation associated with secretory breast carcinoma?
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What is the diagnosis for a periumbilical mass in a healthy woman?
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What cytokeratins are expressed by synovial sarcoma?
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What is the diagnosis for a cystic mass in a 9-year-old boy?
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What is the diagnosis for a large expansile radiolucency involving the right mandible?
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What is the most likely diagnosis for a warty and fungating tumor in the oral cavity?
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Which renal neoplasm is often associated with polycythemia?
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What is the most common association with gastric antral vascular ectasia (GAVE)?
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What is the diagnosis based on histology showing tightly packed tumor nodules?
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What is the most likely diagnosis for a soft tissue mass in a 40-year-old man?
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What is a lymphoma with CD20-dim, CD19-positive, CD5-positive, and CD23-positive characteristics?
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What is this lesion in the lung that exhibits consistent cytogenetic abnormalities?
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What is the diagnosis for a rapidly enlarging nodule with crater-like architecture?
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Which condition would NOT be included in the histologic differential diagnosis of subepidermal infiltrates?
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What is the most likely etiology of anemia in a patient with red cell aplasia secondary to parvovirus infection?
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Which tumor is characterized by a prominent component of irregularly distributed ganglion cells?
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What is the diagnosis for a renal mass found in a patient with flank pain?
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What is the association with fundic gland polyps in familial adenomatous polyposis (FAP)?
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What mutations are commonly associated with sporadic fundic gland polyps?
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What is the most likely diagnosis of the nodule found in the subtotal parotidectomy specimen?
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What is a good negative stain for nephrogenic adenoma?
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What syndrome describes multiple skeletal metastases in neuroblastoma?
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Which enzyme deficiency is most common in hereditary erythrocyte disorders of the glycolytic pathway?
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What deposit is typically seen in gout?
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What diagnosis matches the presentation of a 1-week-old boy with a violaceous plaque on the upper back?
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What is commonly mutated in pancreatic adenocarcinoma?
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What cytogenetic abnormality is associated with a favorable prognosis in B-cell acute lymphoblastic leukemia?
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What syndrome is characterized by necrotizing granulomas?
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What mutation is prevalent in the tall cell variant of papillary thyroid carcinoma?
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Which of the following is least likely to cause centrilobular hepatocyte necrosis?
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What is the diagnosis of a tumor composed predominantly of lobules of hyaline cartilage?
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What is the likely diagnosis for a lesion showing immature trabeculae in a fibrous stroma?
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What is characterized by smooth gelatinous lesions on heart valves in patients with end-stage malignancy?
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What tumor is characterized histologically by bland oval spindle cells with maloriented trabeculae?
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What type of renal tumor is associated with TFE3 and TFE gene alterations?
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What is the diagnosis for a 5-month-old girl with profound hypotonia based on muscle findings?
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In the setting of a renal mass, what lesion is generally negative for PAX-8?
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What is the cytogenetic abnormality associated with extranodal NK/T-cell lymphoma?
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What renal tumor is associated with clear cells and HMB45 positivity?
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What is the diagnosis for an ill-defined radiolucent and radiopaque lesion of the proximal humerus in an athlete?
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What viral infection is associated with multicentric Castleman's disease?
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What is the distribution of multiple enchondromas in Ollier's disease?
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Which of the following conditions does not lead to hypersensitivity pneumonitis?
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What percentage of basal-like cancers are triple negative?
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What is the diagnosis of differentiated vulvar intraepithelial neoplasia (VIN)?
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Where is the CDKN2A gene located?
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What mutation in which gene is responsible for Wilson disease?
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Are squamous markers positive or negative in thymomas?
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What is a distinguishing immunophenotypic difference between mantle zone and marginal zone lymphoma?
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What is the diagnosis of a lesion in the tibia exhibiting biphasic tumor characteristics?
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Which lesion is least likely to demonstrate squamous metaplasia?
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What is the associated molecular or cytogenetic abnormality in hereditary papillary renal cell carcinoma?
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Which subset of diabetes features amyloid deposition in islets?
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What is the staining pattern classic for light chain amyloidosis?
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What is the diagnosis based on nephrotic syndrome and preserved renal function?
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What gene is recurrently rearranged in follicular lymphoma?
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On serology, how do you distinguish between iron-deficiency anemia and anemia of chronic disease?
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What is the diagnosis of small, uniform spindle cells forming densely packed sheets with hemangiopericytoid vascularity?
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What is associated with progressive transformation of germinal centers?
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What is the diagnosis in a liver biopsy showing portal tract alterations in a patient with elevated serum alkaline phosphatase levels?
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Which finding is not associated with psoriasis vulgaris?
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What are the benign cells in voided urine?
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What is the cause of iron overload distribution after multiple transfusions?
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What is the most appropriate diagnosis for a conventional grade 2 chondrosarcoma?
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What is targeted in graft-versus-host disease of the liver?
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What is the diagnosis for a retroperitoneal mass associated with TFE3 gene rearrangement?
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What conditions are likely associated with synovial chondromatosis?
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What mutation is associated with an adverse prognosis in de novo acute myeloid leukemia (AML)?
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What condition is related to bilateral submandibular gland swellings in a 43-year-old man?
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What is the diagnosis for a lesion in the proximal tibia showing an ill-defined radiolucency?
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Which of the following can cause retinal hemorrhages?
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What is the immunoprofile of Reed-Sternberg cells?
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Which of the following is not a risk factor for squamous cell carcinoma of the bladder?
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Study Notes
Trichoepithelioma
- Characterized by islands of basaloid cells with an onion-skin pattern of fibrocytes.
- Presents with horn cysts and abortive hair follicles; often contains papillary mesenchymal bodies.
- Histology reveals bland-appearing basaloid cells in a dermal setting with occasional small follicular lumens.
Epithelioid Hemangioendothelioma
- Typically multifocal, with sclerotic, fibrotic, or necrotic nodules.
- Endothelial neoplasm that is CD31 positive.
Pleomorphic Adenoma
- Exhibits tubular and ductal structures within a hyaline stroma.
- Highly diverse histology, requiring differential diagnosis consideration.
Adenoid Cystic Carcinoma
- Notable for extensive cribriform architecture, resembling salivary duct carcinoma.
- Presents as a firm, gray tumor in the submandibular gland.
Nesidioblastosis
- A disorder of β-cell function linked with hyperinsulinemic hypoglycemia.
- Frequently observed in large-for-gestational-age male infants from diabetic mothers.
Cervical Cytologic Testing
- Recommend repeat testing after 12 months for women with specific dysplasia findings.
Secondary Syphilis
- Histologically noted for a combination of lichenoid and granulomatous features, with many plasma cells present.
Ductal Adenocarcinoma
- Derived from large prostatic ducts; originally thought to be estrogen-dependent due to initial incorrect hypotheses.
MLL Gene Rearrangement
- Commonly disrupted in infantile precursor B-lymphoblastic leukemia, especially involving t(4;11).
Listeria Infection
- Acute villitis and villous destruction in the placenta indicative of maternal infection.
Clear Cell Renal Cell Carcinoma
- Characterized by loss of the short arm of chromosome 3.
Beta-Catenin Expression
- Bladder adenocarcinoma typically shows cytoplasmic or negative beta-catenin, contrasting with nuclear expression in colonic adenocarcinoma.
Aortic Transection
- Classic injury type due to motor vehicle collisions, related to rapid deceleration leading to aortic laceration.
Cardiac Allograft Vasculopathy
- Involves intimal thickening of blood vessels and is a distinct form of accelerated atherosclerosis in transplant patients.
Non-Ossifying Fibroma
- Benign fibrous lesion of bone, appearing as spindle cells in storiform patterns, distinguished from giant cell tumors.
Bile Duct Adenoma
- Exhibits a dense, hyalinized stroma with medium-sized glands and lacks nuclear pleomorphism.
Secretory Breast Carcinoma
- Involves the translocation t(12;15) and exhibits a distinct hormone profile.
Urachal Remnant Cyst
- Characterized by non-Müllerian epithelium, with an associated increased adenocarcinoma risk.
Cytokeratin Expression
- Synovial sarcoma expresses CK8 and CK18, while adamantinoma of long bones exhibits CK14 and CK19.
Pilomatricoma
- Features basaloid cells and shadow cells with possible dystrophic calcification, resembling hair follicle structures.
Giant Cell Reparative Granuloma
- Composed of fibroblastic stroma with giant cells and hemorrhagic foci, distinguishing features from giant cell tumors.
Verrucous Carcinoma
- A well-differentiated neoplasm with warty configurations, showing no metastatic potential, often linked to smoking history.
Metanephric Adenoma
- Associated with polycythemia; presents as a renal neoplasm.
Gastric Antral Vascular Ectasia (GAVE)
- Commonly linked with various autoimmune conditions but excludes hypercoagulability and monoclonal gammopathy.
Alveolar Soft Part Sarcoma
- Histologically characterized by cell nests separated by a vascular network, with distinctive rhomboidal crystals present.
Giant Cell Tumor of Soft Parts
- Affects soft tissue, presents with multinodular tumors, and often requires surgical intervention due to recurrence rates.
Chronic Lymphocytic Leukemia (CLL)
- Histological features include pale areas rich in specific prolymphocytic cells.
Pulmonary Hamartoma
- Consist of cytogenetic abnormalities, supporting their neoplastic classification.
Keratoacanthoma
- Rapidly enlarging nodule with keratin-filled crater and inflammatory infiltrate composed of neutrophils and eosinophils.
Porphyria Cutanea Tarda
- Histologically shows subepidermal bullae with preservation of dermal papillae, often with characteristic PAS-positive material.
Candida Infection
- Most common etiology of liver abscesses among fungal causes; frequently seen in immunocompromised patients.
Plasma Cell Myeloma
- Diagnosis requires plasmacytosis of ≥10% in bone marrow, indicated by the acronym "CRAB."
Parvovirus Infection
- Associated with red cell aplasia and proliferation of erythroid precursors exhibiting viral inclusions.
Ganglioneuroma
- Characterized by ganglion cells mixed with neural tissue, primarily originating from retroperitoneum or mediastinum.
Sarcomatoid Carcinoma
- Any renal cell carcinoma subtype may develop sarcomatoid features, indicating aggressive transformation.
Fundic Gland Polyp
- In familial adenomatous polyposis (FAP), linked to APC mutations, contrasting with sporadic cases associated with β-catenin mutations.
Nephrogenic Adenoma
- Exhibits papillary proliferation with glandular components, denoting specifically a bladder condition.
Hutchinson Syndrome
- Refers to resultant skeletal metastases in neuroblastoma, commonly seen in specific bone structures.
Pyruvate Kinase Deficiency
- Most prevalent hereditary deficiency in erythrocyte glycolytic enzymes, significant in hemolytic anemia cases.### Enzyme Deficiency
- Common enzyme deficiency in hereditary erythrocyte disorders: Glucose-6-phosphate dehydrogenase (G6PD) deficiency.
Monosodium Urate Crystals
- Gout shows needle-shaped, pale-staining deposits of sodium urate with strong negative birefringence under polarized light.
- Pseudogout presents with calcium pyrophosphate crystals appearing as blunt rods or rhomboid plates with weak positive birefringence.
Subcutaneous Fat Necrosis of the Newborn
- Characterized by needle-shaped clefts in adipose tissue and minimal changes in the epidermis and dermis.
- Common diagnosis for a 1-week-old boy with a violaceous plaque on the back: Subcutaneous fat necrosis.
Pancreatic Adenocarcinoma
- KRAS mutations present in 95% of cases.
- SMAD4 mutations also common; AFP levels typically not elevated in pancreatic adenocarcinoma.
Cytogenetic Abnormality
- Favorable prognosis in B-cell acute lymphoblastic leukemia associated with translocation t(12;21).
Necrotizing Granulomas
- Necrotizing fasciitis is acute and lacks granulomas in histological examination.
- Histology may reveal extensive necrosis, polymorphonuclear cell infiltration, and various bacterial forms, but not necrotizing granulomas.
BRAF Mutations
- Tall cell variant of papillary thyroid carcinoma shows a higher prevalence of extrathyroidal extension (pT3).
- BRAF mutations are commonly found in this tumor type.
Ferrous Sulfate Toxicity
- Excessive dietary ferrous sulfate can cause liver toxicity primarily in the periportal regions.
- Less likely to cause centrilobular hepatocyte necrosis compared to other substances listed.
Periosteal Osteosarcoma
- Characterized by lobules of hyaline cartilage and immature mesenchymal cells.
- Radiographic sign includes widespread, fusiform radiolucency with a "sunburst pattern."
Low-Grade Intramedullary Osteosarcoma
- Appears deceptively benign with immature trabeculae and infiltrative growth.
- Histology differs from fibrous dysplasia due to more cellular and consistent atypia.
Nonbacterial Thrombotic Endocarditis (NBTE)
- Often linked to hypercoagulable states and mucin-producing adenocarcinomas.
- Notably lacks significant valve damage, distinguishing it from infective endocarditis.
Fibrous Dysplasia
- Found in ribs, facial bones, and long bones with a fibrocellular stroma and curvilinear trabeculae.
- No cortical destruction or significant periosteal reaction occurs.
Translocation-Associated Renal Cell Carcinoma
- Associated with TFE gene(s) mutations, notably TFE3 and TFEB.
- TFE3-associated tumors feature clear cells with high nuclear grade and solid-papillary architecture.
Werdnig-Hoffmann Disease
- The most severe form of infantile spinal muscular atrophy (SMA).
- Muscle biopsies show highly atrophic fibers with some hypertrophic myofibers.
Urothelial Carcinoma
- Generally negative for PAX-8, aiding in distinguishing it from collecting duct carcinoma in renal masses.
Extranodal NK/T-Cell Lymphoma
- Associated with EBV positivity; no specific cytogenetic abnormalities identified.
Renal Angiomyolipoma
- Tumors from perivascular epithelioid cells (PEComas) characterized by positivity for HMB45 and Melan-A.
Osteosarcoma
- Histology features malignant osteoid or bone in a lace-like pattern, differentiating from chondroblastoma or chondrosarcoma.
HHV-8
- Associated with multicentric Castleman's disease, particularly in HIV-infected patients; unifocal cases are not linked.
Ollier's Disease
- Multiple enchondromas exhibit unilateral and asymmetric distribution, contrasting with hereditary multiple exostoses.
Silo Filler's Disease
- Resulting from nitrogen dioxide exposure, leading to adult respiratory distress syndrome (ARDS).
Triple Negative Breast Cancer
- 77% of basal-like cancers are triple negative; 71% of these defined by immunohistochemistry express basal-like cancer traits.
Differentiated Vulvar Intraepithelial Neoplasia (VIN)
- Not associated with HPV, most common in older women, and features characteristic histological changes.
CDKN2A Location
- Located on chromosome 9p21.
Wilson Disease
- Caused by mutations in the ATP7B gene, leading to copper accumulation in hepatocytes.
Thymomas
- Positive for CD1a and TdT, squamous markers negative, and c-KIT negative.
Mantle Zone vs. Marginal Zone Lymphoma
- Mantle zone lymphoma is CD5 and Cyclin D1-positive, while marginal zone lymphoma is negative for both.
Adamantinoma
- Indolent tibial lesion, characterized by a biphasic tumor with spindle and basaloid cells.
Squamous Metaplasia
- Adenoid cystic carcinoma is least likely to demonstrate squamous metaplasia among the options provided.
Papillary Renal Cell Carcinoma
- Associated with germ line MET mutations; presents in patients with a family history of kidney cancer.
Type 2 Diabetes
- Amyloid deposition in islets is a distinguishing feature of type 2 diabetes, unlike type 1 diabetes.
Light Chain Amyloidosis
- Classic for intense lambda-restricted glomerular staining with a "smudged" texture.
Gene Rearrangements
- BCL2 is associated with follicular lymphoma, BCL1 with mantle cell lymphoma, and BCL6 commonly found in DLBCL.
Iron-Deficiency Anemia vs. Anemia of Chronic Disease
- Differentiated by serum ferritin and TIBC levels: Low ferritin and high TIBC in iron-deficiency anemia; high ferritin and low TIBC in anemia of chronic disease.
High-Grade Monophasic Synovial Sarcoma
- Composed of small, uniform spindle cells with distinctive hemangiopericytoid vascularity.
Progressive Transformation of Germinal Centers
- Associated with reactive lymphadenopathy and occasionally with nodular lymphocyte predominant Hodgkin lymphoma.
Primary Sclerosing Cholangitis
- Associated with ulcerative colitis; characterized by specific histological findings in liver biopsies.
Psoriasis Vulgaris
- Characterized by findings such as confluent parakeratosis, but not typically associated with a band-like mononuclear infiltrate.
Benign Renal Tubular Cells
- Appear in urine as small, cuboidal epithelial cells with pyknotic nuclei, indicating renal health.
Transfusional Iron Overload
- Results from multiple prior transfusions leading to specific iron distribution seen in Prussian blue iron stains.
Conventional Grade 2 Chondrosarcoma
- Shows continuous sheets of atypical round or spindle-shaped cells in a myxoid stroma, indicating moderate differentiation.### Chondrosarcoma
- Grade 1 chondrosarcoma features well-differentiated lobules of cartilage, mimicking normal hyaline cartilage.
- Grade 2 chondrosarcoma presents a more diffuse structure with moderate differentiation and contains spindle-shaped and lacunar cells.
- Stroma in grade 1 is chondroid; in grade 2, it is myxoid.
Interlobular Bile Ducts in Liver Biopsy
- Dysmorphism assessment in interlobular bile ducts includes attenuation and elongation, altered nuclear polarity, and intraepithelial lymphocytes.
- GVHD (graft-versus-host disease) rarely affects central veins; involves portal vein endotheliitis less frequently than liver allograft rejection.
- Arterioles are typically not targeted during GVHD.
PEComa and Cytogenetic Alterations
- TFE3 gene rearrangement occurs in some PEComa cases, revealing a nested or alveolar growth similar to alveolar soft part sarcoma.
- Tumor cells express SMA and HMB45; negative for pankeratin, desmin, S100 protein, CD34, and myogenin.
Synovial Chondromatosis/Osteochondromatosis
- Condition characterized by multiple nodules of bone and cartilage within the synovium, forming villi containing fibroadipose tissue.
- Nodules undergo partial ossification; their cartilaginous surface is covered by synoviocytes.
FLT3 Mutation in AML
- FLT3 mutations indicate a poor prognosis in acute myeloid leukemia (AML) with a normal karyotype, particularly with wild type NPM1.
- In contrast, NPM1 mutations usually signal a favorable outcome when FLT3 is unmutated.
- Biallelic CEBPA mutations also predict favorable prognoses in AML.
Chronic Sclerosing Sialadenitis
- Both chronic sclerosing sialadenitis and eosinophilic angiocentric fibrosis are conditions related to IgG4 affecting the head and neck.
- Other IgG4-related conditions include Mikulicz disease and Riedel thyroiditis.
Myxoid Chondrosarcoma
- Characterized by small monotonous cells in sheets and cords amidst a myxoid stroma.
Retinal Hemorrhages in Infants
- Retinal hemorrhages from violent infant shaking are located at the back of the eye.
- Causes of hemorrhages include aggressive resuscitation, manual strangulation, vigorous Valsalva maneuver, and neck ligatures, but not violent shaking.
Reed-Sternberg Cells Immunoprofile
- Reed-Sternberg cells express CD15 and CD30 but are negative for LCA (leukocyte common antigen) and EMA (epithelial membrane antigen).
Squamous Metaplasia of the Bladder
- Nonkeratinizing squamous metaplasia is common in the bladder trigone, especially in women, and is considered a normal finding.
- Risk factors for squamous cell carcinoma of the bladder include Schistosoma infection, bladder stones, and indwelling catheters, but not nonkeratinizing squamous metaplasia.
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Explore detailed flashcards on trichoepithelioma, focusing on its cellular characteristics such as basaloid cells and fibrocyte patterns. This quiz highlights key histological features and emphasizes the significance of papillary mesenchymal bodies in relation to hair follicles. Enhance your understanding of dermatopathology with these essential study materials.
