PRIMARY ORBITAL LIPOSARCOMA IN LI-FRAUMENI CANCER FAMILY SYNDROME:A CASE REPORT

Luigi Caradonna, Domenico Corradi, Roberta Gobbi, Enrico Sesenna, Tito Poli, Francesco Laganà, Luigi Caradonna

Risultato della ricerca: Article

8 Citazioni (Scopus)

Abstract

Aims and background: The aim of this study was to describe a case of primary orbital liposarcoma in Li-Fraumeni syndrome. Methods and study design: In July 1998 a 20-year-old woman with a histological diagnosis of orbital myxoid liposarcoma underwent surgical treatment in our department. Since the patient's family pedigree met the clinical criteria for the diagnosis of LFS, molecular analysis was performed, which resulted in a molecular profile consistent with Li-Fraumeni syndrome. Results: The patient underwent orbital exenteration extended to the upper eyelid; surgical reconstructive steps were performed to permit placement of an orbital prosthesis. Two years after primary surgery the patient underwent a quadrantectomy with lymphadenectomy of the right axilla because of the presence of a nodule of 1.5 cm in diameter in the upper-lateral quadrant of the right breast. One year after the last surgery, the patient is disease free. Conclusion: The diagnosis of an orbital malignancy in a young patient with a family history of cancer should suggest the presence of an underlying genetic disorder like LFS; with molecular analysis we can now determine the genetic disorder and the exact location of the mutation, and also obtain important prognostic data using specific cellular markers. More prognostic information increases the chances of adequate personalized treatment.
Lingua originaleEnglish
pagine (da-a)96-100
Numero di pagine5
RivistaTumori
Volume91
Stato di pubblicazionePublished - 2005

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Liposarcoma
Li-Fraumeni Syndrome
Inborn Genetic Diseases
Neoplasms
Myxoid Liposarcoma
Axilla
Eyelids
Pedigree
Lymph Node Excision
Prostheses and Implants
Breast
Mutation
Therapeutics

All Science Journal Classification (ASJC) codes

  • Cancer Research

Cita questo

Caradonna, L., Corradi, D., Gobbi, R., Sesenna, E., Poli, T., Laganà, F., & Caradonna, L. (2005). PRIMARY ORBITAL LIPOSARCOMA IN LI-FRAUMENI CANCER FAMILY SYNDROME:A CASE REPORT. Tumori, 91, 96-100.

PRIMARY ORBITAL LIPOSARCOMA IN LI-FRAUMENI CANCER FAMILY SYNDROME:A CASE REPORT. / Caradonna, Luigi; Corradi, Domenico; Gobbi, Roberta; Sesenna, Enrico; Poli, Tito; Laganà, Francesco; Caradonna, Luigi.

In: Tumori, Vol. 91, 2005, pag. 96-100.

Risultato della ricerca: Article

Caradonna, L, Corradi, D, Gobbi, R, Sesenna, E, Poli, T, Laganà, F & Caradonna, L 2005, 'PRIMARY ORBITAL LIPOSARCOMA IN LI-FRAUMENI CANCER FAMILY SYNDROME:A CASE REPORT', Tumori, vol. 91, pagg. 96-100.
Caradonna L, Corradi D, Gobbi R, Sesenna E, Poli T, Laganà F e altri. PRIMARY ORBITAL LIPOSARCOMA IN LI-FRAUMENI CANCER FAMILY SYNDROME:A CASE REPORT. Tumori. 2005;91:96-100.
Caradonna, Luigi ; Corradi, Domenico ; Gobbi, Roberta ; Sesenna, Enrico ; Poli, Tito ; Laganà, Francesco ; Caradonna, Luigi. / PRIMARY ORBITAL LIPOSARCOMA IN LI-FRAUMENI CANCER FAMILY SYNDROME:A CASE REPORT. In: Tumori. 2005 ; Vol. 91. pagg. 96-100.
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abstract = "Aims and background: The aim of this study was to describe a case of primary orbital liposarcoma in Li-Fraumeni syndrome. Methods and study design: In July 1998 a 20-year-old woman with a histological diagnosis of orbital myxoid liposarcoma underwent surgical treatment in our department. Since the patient's family pedigree met the clinical criteria for the diagnosis of LFS, molecular analysis was performed, which resulted in a molecular profile consistent with Li-Fraumeni syndrome. Results: The patient underwent orbital exenteration extended to the upper eyelid; surgical reconstructive steps were performed to permit placement of an orbital prosthesis. Two years after primary surgery the patient underwent a quadrantectomy with lymphadenectomy of the right axilla because of the presence of a nodule of 1.5 cm in diameter in the upper-lateral quadrant of the right breast. One year after the last surgery, the patient is disease free. Conclusion: The diagnosis of an orbital malignancy in a young patient with a family history of cancer should suggest the presence of an underlying genetic disorder like LFS; with molecular analysis we can now determine the genetic disorder and the exact location of the mutation, and also obtain important prognostic data using specific cellular markers. More prognostic information increases the chances of adequate personalized treatment.",
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AU - Caradonna, Luigi

AU - Corradi, Domenico

AU - Gobbi, Roberta

AU - Sesenna, Enrico

AU - Poli, Tito

AU - Laganà, Francesco

AU - Caradonna, Luigi

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N2 - Aims and background: The aim of this study was to describe a case of primary orbital liposarcoma in Li-Fraumeni syndrome. Methods and study design: In July 1998 a 20-year-old woman with a histological diagnosis of orbital myxoid liposarcoma underwent surgical treatment in our department. Since the patient's family pedigree met the clinical criteria for the diagnosis of LFS, molecular analysis was performed, which resulted in a molecular profile consistent with Li-Fraumeni syndrome. Results: The patient underwent orbital exenteration extended to the upper eyelid; surgical reconstructive steps were performed to permit placement of an orbital prosthesis. Two years after primary surgery the patient underwent a quadrantectomy with lymphadenectomy of the right axilla because of the presence of a nodule of 1.5 cm in diameter in the upper-lateral quadrant of the right breast. One year after the last surgery, the patient is disease free. Conclusion: The diagnosis of an orbital malignancy in a young patient with a family history of cancer should suggest the presence of an underlying genetic disorder like LFS; with molecular analysis we can now determine the genetic disorder and the exact location of the mutation, and also obtain important prognostic data using specific cellular markers. More prognostic information increases the chances of adequate personalized treatment.

AB - Aims and background: The aim of this study was to describe a case of primary orbital liposarcoma in Li-Fraumeni syndrome. Methods and study design: In July 1998 a 20-year-old woman with a histological diagnosis of orbital myxoid liposarcoma underwent surgical treatment in our department. Since the patient's family pedigree met the clinical criteria for the diagnosis of LFS, molecular analysis was performed, which resulted in a molecular profile consistent with Li-Fraumeni syndrome. Results: The patient underwent orbital exenteration extended to the upper eyelid; surgical reconstructive steps were performed to permit placement of an orbital prosthesis. Two years after primary surgery the patient underwent a quadrantectomy with lymphadenectomy of the right axilla because of the presence of a nodule of 1.5 cm in diameter in the upper-lateral quadrant of the right breast. One year after the last surgery, the patient is disease free. Conclusion: The diagnosis of an orbital malignancy in a young patient with a family history of cancer should suggest the presence of an underlying genetic disorder like LFS; with molecular analysis we can now determine the genetic disorder and the exact location of the mutation, and also obtain important prognostic data using specific cellular markers. More prognostic information increases the chances of adequate personalized treatment.

KW - liposarcoma, Li-Fraumeni syndrome, orbital surgery.

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