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C.T. Zoon-Besselink
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P2.04 - Poster Session with Presenters Present (ID 466)
- Event: WCLC 2016
- Type: Poster Presenters Present
- Track: Mesothelioma/Thymic Malignancies/Esophageal Cancer/Other Thoracic Malignancies
- Presentations: 1
- Moderators:
- Coordinates: 12/06/2016, 14:30 - 15:45, Hall B (Poster Area)
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P2.04-039 - Long-Term Risk of Recurrence in Benign Pleural Solitary Fibrous Tumors: A Single Institution Review (ID 5782)
14:30 - 14:30 | Author(s): C.T. Zoon-Besselink
- Abstract
Background:
Solitary fibrous tumor (SFT) is a rare tumor of submesothelial origin that can occur in the abdomen, extremities, trunk, head, neck and pleura. Pleural SFTs are defined as benign or malignant based on the number of mitoses and the presence of pleomorphism, hemorrhage, or necrosis. There is limited data in the literature regarding the recurrence risk of benign pleural SFT and the need for long-term follow up in these patients.
Methods:
A single institution retrospective chart review was performed on all surgically resected primary pleural SFTs between 1992 and 2015. Preoperative clinical information, pathologic tumor characteristics, and long-term recurrence and survival data were collected.
Results:
There were 29 primary pleural SFTs resected between 1992 and 2015. Patients had a mean age of 60 years and there were 16 men (55%) and 13 women (45%). Fourteen (48%) presented with symptoms, including two patients with paraneoplastic syndromes, and the other 15 tumors (52%) were found incidentally on imaging. There were six giant SFTs defined as size greater than 15 cm, with two of six giant SFTs undergoing preoperative embolization to aid surgical resection. Otherwise, there were no neoadjuvant or adjuvant treatments in any patient. There was no perioperative 30-day mortality (0%). Mean follow up time was 77 months, during which 4 (14%) patients recurred and 21 of 29 patients (72%) were alive at last follow up. Three of the 8 deaths occurred in patients with recurrent disease. Among the 19 benign pleural SFTs, 2 (11%) recurred at 5 and 9 years postoperatively and 2 of the 6 malignant SFTs (33%) recurred at 4 and 15 years postoperatively. Margin status was known in 25 cases, of which 21 (84%) were negative and 4 (16%) were positive. There were no recurrences in patients with known negative margins.
Conclusion:
This study represents one of the largest contemporary single institution reviews of outcomes of pleural SFT. While benign pleural SFTs were less likely to recur than malignant pleural SFTs, benign pleural SFTs with positive or unknown margin status remain at risk for recurrence up to a decade following resection and require ongoing long-term follow up and surveillance imaging.
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P3.01 - Poster Session with Presenters Present (ID 469)
- Event: WCLC 2016
- Type: Poster Presenters Present
- Track: Biology/Pathology
- Presentations: 1
- Moderators:
- Coordinates: 12/07/2016, 14:30 - 15:45, Hall B (Poster Area)
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P3.01-013 - Case Report of Melanotic Schwannoma: A Challenging Diagnosis Made Clear through Genetic Testing (ID 4943)
14:30 - 14:30 | Author(s): C.T. Zoon-Besselink
- Abstract
Background:
Melanotic schwannomas (MS) are tumors associated with the Carney complex of hyperpigmentation, myxomas, and endocrine overactivity. They most frequently arise from spinal nerve roots and present a diagnostic challenge due to their lack of characteristic pathologic features. We present the case of an otherwise healthy 35-year-old man who presented with nocturnal dyspnea and ptosis. Imaging identified a large 8.1 x 9.2 x 8.4 cm mass in the right apical posterior mediastinum. Core biopsy was consistent with melanoma, although no primary site could be identified. The patient underwent complete R0 resection of an encapsulated posterior thoracic inlet mass adherent to the sympathetic chain and apical parietal pleura. Surgical pathology showed nests of large pleomorphic epithelioid cells with prominent nucleoli and abundant intracytoplasmic pigment consistent with the initial diagnosis of melanoma. The actual diagnosis of melanontic schwannoma was made only when the tumor was sent for molecular testing and a rare mutation was identified.
Methods:
Oncogene sequencing (UCSF-Syapse) was performed on surgically resected formalin-fixed and paraffin embedded tumor. Single nucleotide variations, copy number changes, and rearrangements were detected using a hybridization-based enrichment assay of approximately 500 oncogenes commonly implicated in the development of neoplasia. Of the genes assayed, entire coding regions were analyzed in 429 genes with additional analysis of selected introns in 42 genes.
Results:
Based on standard hematoxyalin and eosin (H&E) stains as well as S-100 and Melan-A positivity on immunohistochemistry (IHC) stains, the specimen was originally diagnosed as melanoma. The initial diagnosis was also supported by a Ki-67 proliferative index of 15%. Molecular testing uncovered a rare PRKAR1A mutation inconsistent with melanoma and consistent with melanotic schwannoma. No other mutations were identified. PRKAR1A mutations are known to occur in up to 70% of Carney complex patients but have never been known to occur in melanoma.
Conclusion:
Standard techniques of H&E and IHC staining with their potential to misdiagnose two similar tumor histologies are outdated in the context of 21st century technology. Modern precision medicine and molecular diagnostics enable the clear distinction of histologically similar tumors. The speed and low cost of sequencing technology has advanced to recommend its frequent use in cases such as this where a diagnosis is not entirely clear.
