Extraskeletal myxoid chondrosarcoma involving the Mons Pubis: a case report and review of the...
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Extraskeletal myxoid chondrosarcoma involving the Mons Pubis: a case report and review of the literature. N Cawley, FA Solomonsz. Bassetlaw District General Hospital References 1. McGrory JE, Rock MG, Nascimento AG, Oliveira AM. Extraskeltal Myxoid Chondrosarcoma. Clinical Orthopaedics and related research. 2000:385;185-190 2. SantaCruz MR, Proctor L, Thomas DB, Gehrig PA. Extraskeletal myxoid chondrosarcoma: A report of a gynaecologic case. Gynecologic Oncology. 98(2005) 498-501 3. J Obstet Gynaecol Res. 2011 Jun 9. doi: 10.1111/j.1447-0756.2011.01559.x. [Epub ahead of print] 4..Mendez-Probst CE, Erdeljan P, Castonguay M, Gabril M, Wehril B, Razvi H. Myxoid chondrosarcoma of the scrotum: a case report and review of the literature. CUAJ.Aug 2010:4(4):109-111 5. Sinha S, Howard A, Peach S. Diagnosis and management of soft tissue sarcoma.BMJ 2010;341:c7170 6. Tateishi U, Hasegawa T, Nojima T, Takegami T, Arai Y. MRI Features of Extraskeletal myxoid chondrosarcoma. Skeletal Radiol, 2006 Jan;35(1)27-33. 7. Handa U, Singhai N, Punia RS, Mohan H. Cytological features and differential diagnosis in a case of extraskeletal mesenchymal chondro14. Jakowski JD, Wakely PE. Cytopathology of Extraskeletal Myxoid Chondrosarcoma. Cancer October 25, 2007:111:5 sarcoma: a case report. Acta Cytol. 2009 Nov-Dec;53(6):704-6. 8.. . Jakowski JD, Wakely PE. Cytopathology of Extraskeletal Myxoid Chondrosarcoma. Cancer October 25, 2007:111:5 9. Drilon AD , Popat S , Bhuchar G , D'Adamo DR , Keohan ML , Fisher C , Antonescu CR , Singer S , Brennan MF , Judson I , Maki RG . Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy. Cancer. 2008 Dec 15;113(12):3364-71 10.A. Jemal, R. Siegel, E. Ward, T. Murray, J. Xu, and M. J. Thun, “Cancer statistics, 2007,” CA: A Cancer Journal for Clinicians, vol. 57, no. 1, pp. 43–66, 2007. 11. National Iinstitute for health and clinical excellence. Improving outcomes for people with sarcoma.. March 2006. Further Management She was referred to the regional Sarcoma team, a post-operative CT scan of chest, abdomen and pelvis with contrast revealed no sign of metastatic disease, this was performed approximately one month following excision. The Sarcoma MDT recommended re-excision of the operation site in order to provide adequate margins of excision along with removal of another skin lesion on the lateral aspect of the right upper thigh. Post-operatively the plan was for her to undergo radiotherapy and have follow- up with the regional sarcoma team. Introduction Gynaecologists are often referred women who present with swelling of the vulval area, Sarcoma is often not at the top of the list for differential diagnoses. Earlier diagnosis leads to improved outcomes and less radical surgery 11 . It is important that as gynaecologists we are aware of the diagnostic features of soft tissue sarcomas. Extraskeletal myxoid chondrosarcoma is a rare tumour. Due to there being only two published case reports where it is described to be involving the vulva 2,3 , we felt it was prudent to present our case. Discussion Our case highlights an extremely rare vulval tumour but also raises awareness regarding the identification of soft tissue sarcomas. The NICE sarcoma guidelines states that delays in diagnosis of soft tissue sarcomas are common and that many are discovered incidentally following an excision of a lump, often the initial excision is inadequate and further treatment is required 11 . This is exactly what happened in our case. The UK Department of Health criteria for urgent referral of a soft tissue mass are; a soft tissue mass >5cm, painful lump, a soft tissue lump that is increasing in size, any lump that is deep to the muscle fascia or recurrence of a lump after a previous excision 5 . Therefore whenever a patient presents with a soft tissue mass involving the vulva we should always consider the possibility of a neoplasm in particular a sarcoma especially if there is a history of increasing size, tenderness or it suspected to be fixed deep to the muscle fascia.. Using the above referral criteria on average 1 in 10 cases will be a sarcoma 11 . Clinical Presentation A 45 year old woman presented with a 5 month history of swelling within Mons Pubis. She described a gradual increase in size and discomfort. Examination revealed a mobile 5cm subcutaneous swelling of the mons pubis, mobile in all planes. An ultrasound arranged by her GP found a 4.5x2.5x3.5cm hypoechoic subcutaneous mass which had appearances consistent with a sebaceous cyst. Nine days after her initial referral she underwent excision of the swelling under GA. Intraoperative findings were of a 3cm lipomatous tumour, it was separated from the surrounding tissue by blunt dissection, it appeared to have been removed intact. Histology Report A well-delineated thinly encapsulated poorly differentiated malignant lesion comprising of ovoid to epithelioid appearing cells with well- defined eosiniphilic cytoplasm , small round ovoid to spindly nucleli with prominent single nucleoli. A focal breach of the capsule was noted so excision was considered incomplete. The Extraskeletal myxoid chondrosarcoma Extra-skeletal myxoid chondrosarcoma is a soft tissue tumour, classified as an intermediate- grade neoplasm. It has a tendency for recurrence and metastasis 1 . It tends to present in the middle-aged 1,9 and commonly originates in the deep tissues of the proximal extremities and limb girdles 4 . Imaging is best by MRI which shows a multi- lobular soft tissue mass often invading extra compartmental bony and vascular structures 6 . Fine tissue aspiration can be used to obtain to a diagnosis in conjunction with radiology 7 . Cytological diagnosis can be made in the presence of a uniform, round to oval cell population often arranged in cords and set in abundant myxoid/chondromyxoid background 8 . The most effective way of managing this tumour is by wide local excision 1 , with aggressive control of local disease 9 . It is relatively resistant to radiotherapy 1 and has a poor response to chemotherapy 9 . It has a high potential for relapse, the mean survival after metastases is 4-5 months 1 .
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Transcript of Extraskeletal myxoid chondrosarcoma involving the Mons Pubis: a case report and review of the...
Extraskeletal myxoid chondrosarcoma involving the Mons Pubis: a case report and review of the literature.
N Cawley, FA Solomonsz. Bassetlaw District General Hospital
References
1. McGrory JE, Rock MG, Nascimento AG, Oliveira AM. Extraskeltal Myxoid Chondrosarcoma. Clinical Orthopaedics and related research. 2000:385;185-1902. SantaCruz MR, Proctor L, Thomas DB, Gehrig PA. Extraskeletal myxoid chondrosarcoma: A report of a gynaecologic case. Gynecologic Oncology. 98(2005) 498-5013. J Obstet Gynaecol Res. 2011 Jun 9. doi: 10.1111/j.1447-0756.2011.01559.x. [Epub ahead of print]4..Mendez-Probst CE, Erdeljan P, Castonguay M, Gabril M, Wehril B, Razvi H. Myxoid chondrosarcoma of the scrotum: a case report and review of the literature. CUAJ.Aug 2010:4(4):109-1115. Sinha S, Howard A, Peach S. Diagnosis and management of soft tissue sarcoma.BMJ 2010;341:c71706. Tateishi U, Hasegawa T, Nojima T, Takegami T, Arai Y. MRI Features of Extraskeletal myxoid chondrosarcoma. Skeletal Radiol, 2006 Jan;35(1)27-33.7. Handa U, Singhai N, Punia RS, Mohan H. Cytological features and differential diagnosis in a case of extraskeletal mesenchymal chondro14. Jakowski JD, Wakely PE. Cytopathology of Extraskeletal Myxoid Chondrosarcoma. Cancer October 25, 2007:111:5sarcoma: a case report. Acta Cytol. 2009 Nov-Dec;53(6):704-6.8.. . Jakowski JD, Wakely PE. Cytopathology of Extraskeletal Myxoid Chondrosarcoma. Cancer October 25, 2007:111:59. Drilon AD, Popat S, Bhuchar G, D'Adamo DR, Keohan ML, Fisher C, Antonescu CR, Singer S, Brennan MF, Judson I, Maki RG. Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy. Cancer. 2008 Dec 15;113(12):3364-7110.A. Jemal, R. Siegel, E. Ward, T. Murray, J. Xu, and M. J. Thun, “Cancer statistics, 2007,” CA: A Cancer Journal for Clinicians, vol. 57, no. 1, pp. 43–66, 2007. 11. National Iinstitute for health and clinical excellence. Improving outcomes for people with sarcoma.. March 2006.
Further ManagementShe was referred to the regional Sarcoma team, a post-operative CT scan of chest, abdomen and pelvis with contrast revealed no sign of metastatic disease, this was performed approximately one month following excision. The Sarcoma MDT recommended re-excision of the operation site in order to provide adequate margins of excision along with removal of another skin lesion on the lateral aspect of the right upper thigh. Post-operatively the plan was for her to undergo radiotherapy and have follow-up with the regional sarcoma team.
IntroductionGynaecologists are often referred women who present with swelling of the vulval area, Sarcoma is often not at the top of the list for differential diagnoses. Earlier diagnosis leads to improved outcomes and less radical surgery11. It is important that as gynaecologists we are aware of the diagnostic features of soft tissue sarcomas.
Extraskeletal myxoid chondrosarcoma is a rare tumour. Due to there being only two published case reports where it is described to be involving the vulva 2,3, we felt it was prudent to present our case.
DiscussionOur case highlights an extremely rare vulval tumour but also raises awareness regarding the identification of soft tissue sarcomas.
The NICE sarcoma guidelines states that delays in diagnosis of soft tissue sarcomas are common and that many are discovered incidentally following an excision of a lump, often the initial excision is inadequate and further treatment is required 11. This is exactly what happened in our case.
The UK Department of Health criteria for urgent referral of a soft tissue mass are; a soft tissue mass >5cm, painful lump, a soft tissue lump that is increasing in size, any lump that is deep to the muscle fascia or recurrence of a lump after a previous excision5. Therefore whenever a patient presents with a soft tissue mass involving the vulva we should always consider the possibility of a neoplasm in particular a sarcoma especially if there is a history of increasing size, tenderness or it suspected to be fixed deep to the muscle fascia.. Using the above referral criteria on average 1 in 10 cases will be a sarcoma11.
Clinical PresentationA 45 year old woman presented with a 5 month history of swelling within Mons Pubis. She described a gradual increase in size and discomfort. Examination revealed a mobile 5cm subcutaneous swelling of the mons pubis, mobile in all planes.
An ultrasound arranged by her GP found a 4.5x2.5x3.5cm hypoechoic subcutaneous mass which had appearances consistent with a sebaceous cyst.
Nine days after her initial referral she underwent excision of the swelling under GA. Intraoperative findings were of a 3cm lipomatous tumour, it was separated from the surrounding tissue by blunt dissection, it appeared to have been removed intact.
Histology ReportA well-delineated thinly encapsulated poorly differentiated malignant lesion comprising of ovoid to epithelioid appearing cells with well-defined eosiniphilic cytoplasm , small round ovoid to spindly nucleli with prominent single nucleoli. A focal breach of the capsule was noted so excision was considered incomplete. The diagnosis confirmed by a sarcoma specialist.
Extraskeletal myxoid chondrosarcomaExtra-skeletal myxoid chondrosarcoma is a soft tissue tumour, classified as an intermediate-grade neoplasm. It has a tendency for recurrence and metastasis 1. It tends to present in the middle-aged 1,9 and commonly originates in the deep tissues of the proximal extremities and limb girdles 4.
Imaging is best by MRI which shows a multi-lobular soft tissue mass often invading extra compartmental bony and vascular structures6. Fine tissue aspiration can be used to obtain to a diagnosis in conjunction with radiology7. Cytological diagnosis can be made in the presence of a uniform, round to oval cell population often arranged in cords and set in abundant myxoid/chondromyxoid background8.
The most effective way of managing this tumour is by wide local excision1, with aggressive control of local disease9. It is relatively resistant to radiotherapy1 and has a poor response to chemotherapy9. It has a high potential for relapse, the mean survival after metastases is 4-5 months1.
