Journal article
Rare case of remission of a patient with small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT) stage IV: Case report.
- Mathey MP Département de chirurgie gynécologique et oncologique, Pr Dr Med A. Feki MD, PhD, HFR, Hôpital Cantonal, 6 chemin des pensionnats, Fribourg 1708, Switzerland; Institut fur med, OnKologie, Inselspital, 3010 Berne, Switzerland. Electronic address: mp491@hotmail.com.
- de Jolinière JB Département de chirurgie gynécologique et oncologique, Pr Dr Med A. Feki MD, PhD, HFR, Hôpital Cantonal, 6 chemin des pensionnats, Fribourg 1708, Switzerland; Institut fur med, OnKologie, Inselspital, 3010 Berne, Switzerland. Electronic address: jean.bouquetdejoliniere@h-fr.ch.
- Major A Département de chirurgie gynécologique et oncologique, Pr Dr Med A. Feki MD, PhD, HFR, Hôpital Cantonal, 6 chemin des pensionnats, Fribourg 1708, Switzerland; Institut fur med, OnKologie, Inselspital, 3010 Berne, Switzerland.
- Conrad B Département de chirurgie gynécologique et oncologique, Pr Dr Med A. Feki MD, PhD, HFR, Hôpital Cantonal, 6 chemin des pensionnats, Fribourg 1708, Switzerland; Institut fur med, OnKologie, Inselspital, 3010 Berne, Switzerland.
- Khomsi F Département de chirurgie gynécologique et oncologique, Pr Dr Med A. Feki MD, PhD, HFR, Hôpital Cantonal, 6 chemin des pensionnats, Fribourg 1708, Switzerland; Institut fur med, OnKologie, Inselspital, 3010 Berne, Switzerland.
- Betticher D Département d'Oncologie médicale, Pr Dr Med D. Betticher MHA, HFR, hôpital Cantonal, 6 chemin des pensionnats, Fribourg 1708, Switzerland; Institut fur med, OnKologie, Inselspital, 3010 Berne, Switzerland.
- Devouassoux M Institut de pathologie Multi-Sites des HCL, Groupements hospitaliers Sud et nord, Pr M. Devouassoux MD, PhD, Centre de biologie et pathologie sud, Bat 3D 69495 Pierre Bénite Cedex, France; Institut fur med, OnKologie, Inselspital, 3010 Berne, Switzerland.
- Feki A Département de chirurgie gynécologique et oncologique, Pr Dr Med A. Feki MD, PhD, HFR, Hôpital Cantonal, 6 chemin des pensionnats, Fribourg 1708, Switzerland; Institut fur med, OnKologie, Inselspital, 3010 Berne, Switzerland.
- 2020-01-25
Published in:
- International journal of surgery case reports. - 2020
English
INTRODUCTION
Small cell carcinoma of the ovary (SCC) is a very rare (less than 1 % of ovarien neoplasia), highly undifferentiated, aggressive malignancy affecting young women and linked to a poor prognosis. Overall survive rate is very low (about 16 %). SCCOHT has recently been shown to be associated with SMARCA4 gene mutations and shows some genetic similarities to malignant rhabdoid tumors (MRT).
PRESENTATION OF CASE
After a reminder of the clinical, histological description of the SCCOHT and concensus about the medical management, we describe the rare case of a 22 years old patient with complete remission after diagnosis of un undifferentiated SCCOHT stage IV treated by conservative surgery and high-doses chemotherapy, 30 months after diagnosis.
DISCUSSION
Thus far, no standard therapy exists for SCCOHT. Treatment modalities are surgery, chemotherapy, radiotherapy and autologous stem cell transplant after high-dose chemotherapy. Research for new treatments includes target therapy.
CONCLUSION
Autologous stem cell transplant after high-dose adjuvant chemotherapy seems to lead to the best survival rates. Invasiveness of the treatment depends on the stage of the disease, age of the patient and her fertility-sparing desire. An international collaboration will be needed to standardise practices due of the small number of patients.
Small cell carcinoma of the ovary (SCC) is a very rare (less than 1 % of ovarien neoplasia), highly undifferentiated, aggressive malignancy affecting young women and linked to a poor prognosis. Overall survive rate is very low (about 16 %). SCCOHT has recently been shown to be associated with SMARCA4 gene mutations and shows some genetic similarities to malignant rhabdoid tumors (MRT).
PRESENTATION OF CASE
After a reminder of the clinical, histological description of the SCCOHT and concensus about the medical management, we describe the rare case of a 22 years old patient with complete remission after diagnosis of un undifferentiated SCCOHT stage IV treated by conservative surgery and high-doses chemotherapy, 30 months after diagnosis.
DISCUSSION
Thus far, no standard therapy exists for SCCOHT. Treatment modalities are surgery, chemotherapy, radiotherapy and autologous stem cell transplant after high-dose chemotherapy. Research for new treatments includes target therapy.
CONCLUSION
Autologous stem cell transplant after high-dose adjuvant chemotherapy seems to lead to the best survival rates. Invasiveness of the treatment depends on the stage of the disease, age of the patient and her fertility-sparing desire. An international collaboration will be needed to standardise practices due of the small number of patients.
- Language
-
- English
- Open access status
- gold
- Identifiers
-
- DOI 10.1016/j.ijscr.2019.11.053
- PMID 31978720
- Persistent URL
- https://sonar.ch/global/documents/166559
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