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Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 19
Published: 01 February 2024
10.55418/9781933477428-01
ISBN-10: 1-933477-42-3
ISBN: 978-1-933477-42-8
... ), Laugier-Hunziger syndrome ( 10 ), and Peutz-Jeghers syndrome ( fig. 1-8 ) ( 11 ). Carney complex includes two subsets. One is referred to as the NAME syndrome, including melanocytic nevi, especially epithelioid blue n evi and lentigines, a trial myxomas, m yxoid soft tissue tumors, and e ndocrine...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 19
Published: 01 February 2024
10.55418/9781933477428-10
ISBN-10: 1-933477-42-3
ISBN: 978-1-933477-42-8
... syndrome, with risk for ocular melanoma, mesothelioma, renal cell carcinoma, and other neoplasms ( Table 10-2 ) ( 11 ). Microscopic features suggestive of a germline mutation include the presence of junctional nests with BAP1 loss and fatty metaplasia of dermal melanocytes. Some BAP1-inactivated nevi show...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 19
Published: 01 February 2024
10.55418/9781933477428-04
ISBN-10: 1-933477-42-3
ISBN: 978-1-933477-42-8
.... Metcalf   JS , Maize   JC . Clark’s nevus . Semin Cutan Med Surg   1999 ; 18 : 43 - 6 . 5. Clark   WH   Jr , Reimer   RR , Greene   M , Ainsworth   AM , Mastrangelo   MJ . Origin of familial malignant melanomas from heritable melanocytic lesions. The “B-K mole syndrome...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 19
Published: 01 February 2024
10.55418/9781933477428-02
ISBN-10: 1-933477-42-3
ISBN: 978-1-933477-42-8
... “satellite nevi,” sometimes widely disseminated throughout the body ( fig. 2-2 ). Multiple satellites (over 20), multiple CMNs, a giant CMN, or a posterior axial location increases the risk of neurocutaneous melanosis (NCM), more recently termed as CMN syndrome ( 8 , 9 ). NCM...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 19
Published: 01 February 2024
10.55418/9781933477428-20
ISBN-10: 1-933477-42-3
ISBN: 978-1-933477-42-8
... melanoma in an infant with congenital melanocytic nevus syndrome and multiple, NRAS and BRAF mutation-negative nodules . Pediatr Dermatol   2018 ; 35 : e281 - 5 . 13. Moore-Olufemi   S , Herzog   C , Warneke   C , et al . Outcomes in pediatric melanoma: comparing prepubertal...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 18
Published: 01 January 2024
10.55418/9781933477367-10
ISBN-10: 1-933477-36-9
ISBN: 978-1-933477-36-7
..., particularly in instances of hyper-estrogen states and in men with Klinefelter syndrome. Breast tissue removed from transgender men, particularly those taking testosterone and related compounds, demonstrates varying degrees of lobular involution, as well as a lower frequency of lesions such as fibroadenoma...
Images
MARFAN <span class="search-highlight">SYNDROME</span>  The myocardium in patients with Marfan <span class="search-highlight">syndrome</span> may exhibi...
Published: 15 November 2023
MARFAN SYNDROME Figure 19-24 MARFAN SYNDROME The myocardium in patients with Marfan syndrome may exhibit both myocyte hypertrophy and interstitial (pericellular-type) fibrosis, mimicking a dilated cardiomyopathy. More about this image found in MARFAN SYNDROME The myocardium in patients with Marfan syndrome may exhibi...
Images
ANTIPHOSPHOLIPID <span class="search-highlight">SYNDROME</span>  A fibrin-rich thrombus is seen within a small-ca...
Published: 15 November 2023
ANTIPHOSPHOLIPID SYNDROME Figure 6-16 ANTIPHOSPHOLIPID SYNDROME A fibrin-rich thrombus is seen within a small-caliber intramyocardial coronary artery. More about this image found in ANTIPHOSPHOLIPID SYNDROME A fibrin-rich thrombus is seen within a small-ca...
Images
NOONAN <span class="search-highlight">SYNDROME</span>  This example from a pediatric patient exhibits marked bive...
Published: 15 November 2023
NOONAN SYNDROME Figure 19-23 NOONAN SYNDROME This example from a pediatric patient exhibits marked biventricular hypertrophy, which manifests as concentric thickening of both the right and left ventricles (RV and LV, respectively). More about this image found in NOONAN SYNDROME This example from a pediatric patient exhibits marked bive...
Images
SYSTEMIC LUPUS ERYTHEMATOSUS AND THE ANTIPHOSPHOLIPID <span class="search-highlight">SYNDROME</span>  A: Lymphocy...
Published: 15 November 2023
SYSTEMIC LUPUS ERYTHEMATOSUS AND THE ANTIPHOSPHOLIPID SYNDROME Figure 21-3 SYSTEMIC LUPUS ERYTHEMATOSUS AND THE ANTIPHOSPHOLIPID SYNDROME A: Lymphocytic myocarditis, typically microfocal, is seen. B: Fibrin microthrombi are seen in both arteries and veins. C: Interstitial (replacement-type) fib... More about this image found in SYSTEMIC LUPUS ERYTHEMATOSUS AND THE ANTIPHOSPHOLIPID SYNDROME A: Lymphocy...
Images
MARFAN <span class="search-highlight">SYNDROME</span>: PHYSICAL FINDINGS  Long, slender fingers mimic the legs of...
Published: 15 November 2023
MARFAN SYNDROME: PHYSICAL FINDINGS Figure 31-1 MARFAN SYNDROME: PHYSICAL FINDINGS Long, slender fingers mimic the legs of a spider, so-called arachnodactyly (left), and there can be joint hypermobility (right). More about this image found in MARFAN SYNDROME: PHYSICAL FINDINGS Long, slender fingers mimic the legs of...
Images
MARFAN <span class="search-highlight">SYNDROME</span>: COMPUTERIZED TOMOGRAPHY  This thin maximum intensity proje...
Published: 15 November 2023
MARFAN SYNDROME: COMPUTERIZED TOMOGRAPHY Figure 31-2 MARFAN SYNDROME: COMPUTERIZED TOMOGRAPHY This thin maximum intensity projection shows dilatation of the ascending aorta (black double-headed arrow). (Courtesy of Dr. P. M. Young, Rochester, MN) More about this image found in MARFAN SYNDROME: COMPUTERIZED TOMOGRAPHY This thin maximum intensity proje...
Images
MARFAN <span class="search-highlight">SYNDROME</span>: AORTIC ANEURYSM  Dilatation is often most extreme in the p...
Published: 15 November 2023
MARFAN SYNDROME: AORTIC ANEURYSM Figure 31-3 MARFAN SYNDROME: AORTIC ANEURYSM Dilatation is often most extreme in the proximal segments and then progresses distally. More about this image found in MARFAN SYNDROME: AORTIC ANEURYSM Dilatation is often most extreme in the p...
Images
MARFAN <span class="search-highlight">SYNDROME</span>: AORTIC ANNULAR DILATATION  The cusps are stretched seconda...
Published: 15 November 2023
MARFAN SYNDROME: AORTIC ANNULAR DILATATION Figure 31-4 MARFAN SYNDROME: AORTIC ANNULAR DILATATION The cusps are stretched secondary to the aortic annular dilatation and no longer centrally coapt, causing valvular regurgitation. More about this image found in MARFAN SYNDROME: AORTIC ANNULAR DILATATION The cusps are stretched seconda...
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MARFAN <span class="search-highlight">SYNDROME</span>: MUCOID EXTRACELLULAR MATRIX ACCUMULATION (MEMA)  Top: Abun...
Published: 15 November 2023
MARFAN SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION (MEMA) Figure 31-5 MARFAN SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION (MEMA) Top: Abundant pools of mucopolysaccharides and glycosaminoglycans deposit within disrupted elastic fibers in a translamellar fashion. Bottom: The pools ma... More about this image found in MARFAN SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION (MEMA) Top: Abun...
Images
MARFAN <span class="search-highlight">SYNDROME</span>: MUCOID EXTRACELLULAR MATRIX ACCUMULATION (MEMA)  A,B: The ...
Published: 15 November 2023
MARFAN SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION (MEMA) Figure 31-6 MARFAN SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION (MEMA) A,B: The basophilic mucopolysaccharide accumulation is evident on hematoxylin and eosin (H&E) (A) and can be seen within disrupted elastic fibers on el... More about this image found in MARFAN SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION (MEMA) A,B: The ...
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MARFAN <span class="search-highlight">SYNDROME</span>: MEDIAL DISSECTION  An intimal tear (black arrow) extends a...
Published: 15 November 2023
MARFAN SYNDROME: MEDIAL DISSECTION Figure 31-8 MARFAN SYNDROME: MEDIAL DISSECTION An intimal tear (black arrow) extends about two thirds of the way through the damaged aortic media (elastic tissue stain). More about this image found in MARFAN SYNDROME: MEDIAL DISSECTION An intimal tear (black arrow) extends a...
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LOEYS-DIETZ <span class="search-highlight">SYNDROME</span>: ARTERIAL TORUOSITY  The branch vessels of the great a...
Published: 15 November 2023
LOEYS-DIETZ SYNDROME: ARTERIAL TORUOSITY Figure 31-9 LOEYS-DIETZ SYNDROME: ARTERIAL TORUOSITY The branch vessels of the great arteries (Ao, aorta; PA, pulmonary artery), such as the right pulmonary artery (RPA) and left pulmonary artery (LPA), are coiled and tortuous, as are the vessels of the ao... More about this image found in LOEYS-DIETZ SYNDROME: ARTERIAL TORUOSITY The branch vessels of the great a...
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LOEYS-DIETZ <span class="search-highlight">SYNDROME</span>: MUCOID EXTRACELLULAR MATRIX ACCUMULATION  A,B: Large ...
Published: 15 November 2023
LOEYS-DIETZ SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION Figure 31-10 LOEYS-DIETZ SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION A,B: Large pools can be seen depositing in a transmural fashion throughout the aorta (A) with elastic fiber loss (B). C,D: Translamellar MEMA is also noted (... More about this image found in LOEYS-DIETZ SYNDROME: MUCOID EXTRACELLULAR MATRIX ACCUMULATION A,B: Large ...