Long-term survival of patients with mismatch repair protein-deficient, high-stage ovarian clear cell carcinoma

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    Abstract

    Aims: Gynaecological cancer patients with germline mutations appear to have a better prognosis than those with sporadic malignancies. Following the observation of long-term survival in a patient with stage III ovarian clear cell carcinoma (CCC) and possible Lynch syndrome (LS), DNA mismatch repair (MMR) protein immunohistochemistry was performed in a series of high-stage CCC and correlated with patient outcomes. Methods and results: Thirty-two consecutive cases of stage III/IV ovarian CCCs accessioned between 1992 and 2015 were examined. The tumours from two patients (6%), including the index case, showed loss of MSH2/MSH6 expression while MLH1/PMS2 staining was retained. The index patient subsequently developed colonic and rectal carcinomas that were also MSH2/MSH6-deficient, while the second patient had a genetically confirmed germline MSH2 mutation. All other tumours showed retained expression of the four MMR proteins. The two patients with MMR protein-deficient tumours were alive 160 months and 124 months following surgery, whereas the median survival of patients with MMR protein-intact CCCs was 11.8 months (75th and 25th percentiles of 8.1 months and 39.3 months, respectively), with 21 patients deceased due to tumour. Conclusions: Larger studies are required but high-stage, MMR protein-deficient CCCs may have a relatively favourable prognosis.

    Original languageEnglish
    Pages (from-to)309-313
    Number of pages5
    JournalHistopathology
    Volume70
    Issue number2
    DOIs
    Publication statusPublished - 1 Jan 2017

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    DNA Mismatch Repair
    Carcinoma
    Survival
    Proteins
    Neoplasms
    Germ-Line Mutation
    Hereditary Nonpolyposis Colorectal Neoplasms
    Immunohistochemistry
    Observation
    Staining and Labeling

    Cite this

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    title = "Long-term survival of patients with mismatch repair protein-deficient, high-stage ovarian clear cell carcinoma",
    abstract = "Aims: Gynaecological cancer patients with germline mutations appear to have a better prognosis than those with sporadic malignancies. Following the observation of long-term survival in a patient with stage III ovarian clear cell carcinoma (CCC) and possible Lynch syndrome (LS), DNA mismatch repair (MMR) protein immunohistochemistry was performed in a series of high-stage CCC and correlated with patient outcomes. Methods and results: Thirty-two consecutive cases of stage III/IV ovarian CCCs accessioned between 1992 and 2015 were examined. The tumours from two patients (6{\%}), including the index case, showed loss of MSH2/MSH6 expression while MLH1/PMS2 staining was retained. The index patient subsequently developed colonic and rectal carcinomas that were also MSH2/MSH6-deficient, while the second patient had a genetically confirmed germline MSH2 mutation. All other tumours showed retained expression of the four MMR proteins. The two patients with MMR protein-deficient tumours were alive 160 months and 124 months following surgery, whereas the median survival of patients with MMR protein-intact CCCs was 11.8 months (75th and 25th percentiles of 8.1 months and 39.3 months, respectively), with 21 patients deceased due to tumour. Conclusions: Larger studies are required but high-stage, MMR protein-deficient CCCs may have a relatively favourable prognosis.",
    keywords = "clear cell carcinoma, lynch syndrome, ovary, prognosis, stage",
    author = "Stewart, {Colin J R} and Bowtell, {David D L} and Doherty, {Dorota A.} and Leung, {Yee C.}",
    year = "2017",
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    Long-term survival of patients with mismatch repair protein-deficient, high-stage ovarian clear cell carcinoma. / Stewart, Colin J R; Bowtell, David D L; Doherty, Dorota A.; Leung, Yee C.

    In: Histopathology, Vol. 70, No. 2, 01.01.2017, p. 309-313.

    Research output: Contribution to journalArticle

    TY - JOUR

    T1 - Long-term survival of patients with mismatch repair protein-deficient, high-stage ovarian clear cell carcinoma

    AU - Stewart, Colin J R

    AU - Bowtell, David D L

    AU - Doherty, Dorota A.

    AU - Leung, Yee C.

    PY - 2017/1/1

    Y1 - 2017/1/1

    N2 - Aims: Gynaecological cancer patients with germline mutations appear to have a better prognosis than those with sporadic malignancies. Following the observation of long-term survival in a patient with stage III ovarian clear cell carcinoma (CCC) and possible Lynch syndrome (LS), DNA mismatch repair (MMR) protein immunohistochemistry was performed in a series of high-stage CCC and correlated with patient outcomes. Methods and results: Thirty-two consecutive cases of stage III/IV ovarian CCCs accessioned between 1992 and 2015 were examined. The tumours from two patients (6%), including the index case, showed loss of MSH2/MSH6 expression while MLH1/PMS2 staining was retained. The index patient subsequently developed colonic and rectal carcinomas that were also MSH2/MSH6-deficient, while the second patient had a genetically confirmed germline MSH2 mutation. All other tumours showed retained expression of the four MMR proteins. The two patients with MMR protein-deficient tumours were alive 160 months and 124 months following surgery, whereas the median survival of patients with MMR protein-intact CCCs was 11.8 months (75th and 25th percentiles of 8.1 months and 39.3 months, respectively), with 21 patients deceased due to tumour. Conclusions: Larger studies are required but high-stage, MMR protein-deficient CCCs may have a relatively favourable prognosis.

    AB - Aims: Gynaecological cancer patients with germline mutations appear to have a better prognosis than those with sporadic malignancies. Following the observation of long-term survival in a patient with stage III ovarian clear cell carcinoma (CCC) and possible Lynch syndrome (LS), DNA mismatch repair (MMR) protein immunohistochemistry was performed in a series of high-stage CCC and correlated with patient outcomes. Methods and results: Thirty-two consecutive cases of stage III/IV ovarian CCCs accessioned between 1992 and 2015 were examined. The tumours from two patients (6%), including the index case, showed loss of MSH2/MSH6 expression while MLH1/PMS2 staining was retained. The index patient subsequently developed colonic and rectal carcinomas that were also MSH2/MSH6-deficient, while the second patient had a genetically confirmed germline MSH2 mutation. All other tumours showed retained expression of the four MMR proteins. The two patients with MMR protein-deficient tumours were alive 160 months and 124 months following surgery, whereas the median survival of patients with MMR protein-intact CCCs was 11.8 months (75th and 25th percentiles of 8.1 months and 39.3 months, respectively), with 21 patients deceased due to tumour. Conclusions: Larger studies are required but high-stage, MMR protein-deficient CCCs may have a relatively favourable prognosis.

    KW - clear cell carcinoma

    KW - lynch syndrome

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