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Slide Seminar 19 - Pediatric Oncologic Pathology
Wednesday, September 20, 2006 14:00 - 17:30
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Moderators:
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Dr. Tony Bourne and Dr. Denis Benjamin
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Disclosure:
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In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Slide Seminar) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. Faculty members for this Slide Seminar have indicated they have no disclosures to make.
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Clinical histories are displayed below.
Click on the case numbers to display the text and references for each case.
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- Disseminated Intravascular Carcinomatosis
Submitted by: Dr. Marian Malone
14 year old girl. Presented with expressive aphasia. MRI showed white matter changes. CXR and CAT scan: marked mediastinal lymphadenopathy and pleural effusion. Thoracoscopic biopsy.
Case 1 - Slide 1
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- Multicystic Ovaries Due to Hypothyroidism
Submitted by: Dr. Ronald Jaffe
A 9 year-old girl presented with a 2-year history of abdominal pain, increasing abdominal girth and fatigue. The physical examination was unremarkable, Tanner stage 0, but she had palpable abdominal masses. A CT scan revealed cystic masses arising from the pelvis to the level of the umbilicus, multiloculated, multicystic and ovarian in origin. The cysts were decompressed laparoscopically and the cyst wall biopsied. The cytology was benign and the cysts were diagnosed as "luteinized follicular cysts", with the note that since they were large and bilateral, a non-ovarian endocrine effect was possible. Within 2 months, the ovarian cysts recurred, larger than before and were removed laparoscopically. The cysts were signed out as "multiple luteinized cysts with a prominent granulosa cell layer", but an expert consultation was sought because of the clinical concern around their rapid re-growth. The consultant diagnosed "multicystic juvenile granulosa cell tumors", the in-house diagnosis was revised accordingly. The slide is from this procedure. A bilateral salpingo-oophorectomy was done, 4 months after first presentation. The diagnosis was "cystic ovaries with multicystic granulosa cell tumor", with a note that suggested that the granulosa cell tumors were on the background of (non-neoplastic) cystic ovaries and that no extra-ovarian spread was present. The consultant confirmed his original diagnosis and agreed that there were also benign cysts. She was treated with Cisplantin, VP-16 and Bleomycin for 6 months and one year later, is well without evidence of disease.
Case 2 - Slide 1
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- Follicular Dendritic Cell Sarcoma/tumour, Low to Intermediate Grade
Submitted by: Dr. David Parham
This 12 year-old Caucasian girl presented to her local pediatrician with swelling above her right clavicle. Examination revealed a large, non-tender, freely movable soft tissue mass. CT scans revealed a 2.8 x 3.0 x 5.0 cm mass in the right supraclavicular region. There was an indistinct margin of fat. The right clavicle showed no evidence of bone destruction. A 1.5 cm lymph node was also noted in the right axilla, but no other lesions were found in the thorax or abdomen. Following an incisional biopsy, an excision was performed.
Case 3 - Slide 1
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- Poorly Differentiated Malignant Neoplasm Showing a "BRD4-variant" Translocation
Submitted by: Dr. Sara Vargas
This previously healthy girl presented at age 23 months with a 3-day history of shortness of breath. Mild subcostal and intercostal retractions were observed. There were bronchovesicular breath sounds on the right side accompanied by inspiratory and expiratory wheezes. A 0.5-cm firm mobile subclavicular lymph node was palpable. Chest x-rays showed complete opacification of the right hemithorax and a left mediastinal shift, and a CT scan demonstrated a large mass in the right hemithorax and a pleural effusion.
On hospital day #2, open biopsy and debulking were performed via median sternotomy, during which the tumor was noted to involve the right lung and encase the trachea. Within several days the patient developed superior vena cava (SVC) syndrome and increasing respiratory and ventilatory failure. On hospital day #9, she underwent an emergent tumor resection which included a right pneumonectomy further mediastinal debulking and a right pneumonectomy. Recrudescent growth led to recurrent SVC syndrome. Chemotherapy (ifosfamide, adriamycin, and vincristine), begun on hospital day #40, was complicated by pancytopenia, and in the setting of worsening ventilatory failure, the patient died on hospital day #49. An autopsy was declined.
Case 4 - Slide 1
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- MEN 2B
Submitted by: Dr. C. W. Chow
This male baby was born at term by lower segment Caesarian section after breech labour. He was referred from a peripheral hospital to the Royal Children's Hospital on day 13 following abdominal distension, vomiting and 10% loss in weight. Suction rectal biopsy was performed. Acetylcholinesterase showed a prominent submucosal plexus, but the coarse discrete cholinergic nerve fibres in the muscularis mucosae typical of Hirschsprung's disease were not seen.
Case 5 - Slide 1
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- Intra-neural Synovial Sarcoma with SYT-SSX Translocation. Grade 2 Histoprognostic (According to FNCLCC)
Submitted by: Dr. Liliane Boccon-Gibod
Girl aged 13 y (DOB 5/5/92). Mass present since October 2004, developed in the posterior aspect of the right arm, in the brachial biceps, adherent to ulnar nerve. In December, 2005, at MRI the mass measures 39 x 20 mm and is well limited. Biopsy in February, 2006.
Case 6 - Slide 1
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- Central Giant Cell Granuloma
Submitted by: Dr. Jane Dahlstrom
This is a 15 year old boy who presented with a less than 6 months history of rapidly growing right sided palatal mass and loose teeth.
Case 7 - Slide 1
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- Neurocutaneous Melanosis (NCM)
Submitted by: Dr. Miguel Reyes-Múgica
The patient, a 3 year-old male was admitted to hospital with abdominal distention and a right pleural effusion. He had been followed for a number of years as an outpatient in the neurology clinic with persistent complex partial seizures (vide infra).
At surgery a moderate quantity of blood tinged cloudy ascitic fluid was present. Soft, white to tan-white, friable velvety tumor nodules and patches were plastered on the peritoneal surface of all the abdominal organs and omentum. A few of the nodules were variably pigmented, from brown to dark grey. Representative portions of tumor were resected for pathological examination. A right thoracostomy tube was inserted and some of the pleural fluid was submitted for cytocentrifuge preparation and cytologic examination.
Past history:
This patient first presented at the age of 4 months with seizures and opisthotonus, and was found to have severe hydrocephalus and a posterior fossa cyst. The latter was associated with atrophy of the medial portions of the cerebellar hemispheres. Following clinical stabilization, a ventriculo-peritoneal shunt was inserted with some minor improvement in central nervous system function. However, his seizures persisted and a year later he was operated on for a tethered cord. At the time of this surgery the neurosurgeon noted darkly pigmented meninges. Pathological examination was consistent with meningeal melanosis. His epilepsy was treated with a variety of anti-seizure medications. His neurological development was moderately delayed.
The infant was the product of a normal pregnancy and delivery. At birth, a large Mongolian spot and multiple cutaneous brown, pigmented macules of varying size and shape were noted. Approximately twenty lesions were present, a few up to 10 cm in maximum dimension. These were clinically followed by a pediatric dermatologist and showed no significant changes over time. The child has an oldest sister who is healthy. There was no pertinent family history.
Case 8 - Slide 1
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- Unusual Presentation of Childhood Papillary Thyroid Carcinoma
Submitted by: Dr. Ronald R. de Krijger
No clinical history submitted.
Case 9 - Slide 1
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