Locally invasive recurrence or metastasis of pheochromocytoma into the liver?-clinicopathological challenges
© 2022. The Author(s)..
Pheochromocytomas (PCC) are rare and functional neuroendocrine tumors developing from adrenal chromaffin cells. Predicting malignant behavior especially in the absence of metastasis can be quite challenging even in the era of improved understanding of the molecular mechanisms involved in PCCs. Currently, two histopathological grading systems Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) score are used in clinical practice, but these are subject to significant interobserver variability. Some of the most useful clinical factors associated with malignancy are large size ([4-5 cm), and genetic features such as presence of SDHB germline mutations. Local invasion is uncommon in PCC and metastasis seen in 10 to 17% but higher in germline mutations and when this occurs management can be challenging. Here, we report on a case with challenges faced by the pathologist and clinicians alike in diagnosis and management of PCC recurrence.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:20 |
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| Enthalten in: |
World journal of surgical oncology - 20(2022), 1 vom: 11. Nov., Seite 360 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Tang, Sarah S [VerfasserIn] |
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| Links: |
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| Themen: |
Adrenalectomy |
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| Anmerkungen: |
Date Completed 15.11.2022 Date Revised 17.11.2022 published: Electronic Citation Status MEDLINE |
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| doi: |
10.1186/s12957-022-02817-6 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM348825528 |
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| 245 | 1 | 0 | |a Locally invasive recurrence or metastasis of pheochromocytoma into the liver?-clinicopathological challenges |
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| 500 | |a Date Revised 17.11.2022 | ||
| 500 | |a published: Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2022. The Author(s). | ||
| 520 | |a Pheochromocytomas (PCC) are rare and functional neuroendocrine tumors developing from adrenal chromaffin cells. Predicting malignant behavior especially in the absence of metastasis can be quite challenging even in the era of improved understanding of the molecular mechanisms involved in PCCs. Currently, two histopathological grading systems Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) score are used in clinical practice, but these are subject to significant interobserver variability. Some of the most useful clinical factors associated with malignancy are large size ([4-5 cm), and genetic features such as presence of SDHB germline mutations. Local invasion is uncommon in PCC and metastasis seen in 10 to 17% but higher in germline mutations and when this occurs management can be challenging. Here, we report on a case with challenges faced by the pathologist and clinicians alike in diagnosis and management of PCC recurrence | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Adrenalectomy | |
| 650 | 4 | |a Chemotherapy | |
| 650 | 4 | |a Pheochromocytoma | |
| 650 | 4 | |a Radiotherapy | |
| 700 | 1 | |a Lee, James W K |e verfasserin |4 aut | |
| 700 | 1 | |a Wijerethne, Sujith |e verfasserin |4 aut | |
| 700 | 1 | |a Iyer, Shridhar Ganpathi |e verfasserin |4 aut | |
| 700 | 1 | |a Hue, Susan |e verfasserin |4 aut | |
| 700 | 1 | |a En, Nga Min |e verfasserin |4 aut | |
| 700 | 1 | |a Parameswaran, Rajeev |e verfasserin |4 aut | |
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