2017 Annual Meeting

Evening Specialty Conference
Pediatric Pathology

Hereditary Cancer Predisposition in Children

March 5 2017, 7:30pm to 9:30pm

Moderator:
Megan K. Dishop, MD
Panelists:
Ashley Hill, MD - Kyle Kurek, MD - Alexander Judkins, MD - Cristina Antonescu, MD

Pre-Meeting Materials

Case 1

Faculty:
Ashley Hill, MD

Clinical History:

The patient is a 14 year-old girl with a right ovarian mass measuring 7.8 x 7.2 x 6.0 cm.

Morphological Findings (click to enlarge):

Figure 1Right ovarian mass

Case 2

Faculty:
Kyle Kurek, MD

Clinical History:

Mass removed from the dorsum of the left hand of a 4 year-old boy. The mass was first noticed at 1 year of age, and has slowly increased in size, with more rapid growth and associated pain noted in the last 6 months. The mass was firm with focal red-blue surface discoloration. On MRI evaluation, it appeared solid (3 x 3 x 4 cm) and demonstrated heterogeneous hyperintense signal with diffuse enhancement. It appeared to originate within the third intermetacarpal space and extensively involved both dorsal and palmar interosseous muscles and encased the extensor and flexor tendons. The patient was the product of an uneventful pregnancy, born to healthy, non-consanguineous parents, and has two healthy siblings. At birth, he was noted to have a fairly large head (90th percentile), a subcutaneous soft tissue mass on the left flank, and a large congenital pigmented nevus. He had mild hypotonia at birth, and remains "very clumsy," but is otherwise reaching his cognitive developmental milestones.

Morphological Findings (click to enlarge):


Case 3

Faculty:
Alexander Judkins, MD

Clinical History:

4 year old male infant who presents with a history of 6 weeks of early morning vomiting and bilateral horizontal gaze pals. Birth history: born to 23 year old G1P0 mother @ 40 weeks by emergent C-section, no NICU stay Family history: patient is one of two children; sister, healthy at 6 months of age; mother died of cancer at age 27 years (stomach, possibly other organs) 5 months prior to this presentation. Imaging studies identified multifocal tumor was not biopsied and treated as an infiltrative low grade glioma. After one month of treatment the patient's symptoms were worse, tumor had continued to grow, and a biopsy was performed.

Morphological Findings (click to enlarge):

Figure 1
Figure 2
Figure 3

Case 4

Faculty:
Cristina Antonescu, MD

Clinical History:

17 year-old man presented to the ER with an acute abdomen thought to represent an acute appendicitis. Upon intra-abdominal laparoscopic exploration a 4.5 cm gastric mass was identified and a distal gastric resection was performed.

Pertinent Laboratory Data:

Representative H&E images show a cellular but monomorphic spindle to epithelioid neoplasm arranged in solid to reticular patterns embedded in a variably myxoid stroma. The tumor was associated with a high mitotic activity.

Morphological Findings (click to enlarge):

Figure 1
Figure 2

Meetings

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