PRIMARY SOFT TISSUE SARCOMAS OF THE CRANIOFACIAL AREA: A CASE SERIES AND A REVIEW OF LITERATURE
Authors:
Hatzopoulos G, Papakosta V, Shihada A, Kardara-Bellou M, Mpalakas A, Lefantzis N, Vasiliou S
Affiliation:
Department of Oral and Maxillofacial Surgery, Attikon Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Doi: 10.54936/haoms242p44
ABSTRACT:
Objectives: TSoft tissue sarcomas of craniofacial area constitute very rare malignancies with histological and clinical diversity. This study aims to present our clinical experience on craniofacial soft tissue sarcomas in pre-COVID and post-COVID era. A literature review is also conducted with emphasis to epidemiology, diagnosis, treatment and prognosis.
Materials and Methods: We reviewed the cases of 7 patients with craniofacial soft tissue sarcomas that were treated in our Clinic the period 2012-2022. We registered the following information: sarcoma type, diagnostic criteria, disease status at presentation, treatment, outcome and differences in pre- and post-covid era.
Results: There were 6 men and 1 woman with a median age of 56. These cases included: 1 scalp pleiomorphic sarcoma, 1 parotid gland liposarcoma, 1 supraorbital myofibroblastic sarcoma, 1 paranasal Malignant Peripheral Nerve Sheath Tumor, 1 paranasal synovial sarcoma, 1 paranasal angiosarcoma and 1 dermatofibrosarcoma. The diagnosis was histological in all cases. Most cases presented with AJCC stage II disease. Treatment was multidisciplinary. Extensive surgical excision was used in all cases, except 1 due to patient’ s denial. Surgical margins were microscopically positive in 3 patients. Induction chemotherapy was used in the case of MPNST and postoperative chemoradiotherapy in the case of dermatofibrosarcoma. At last follow up 3 patients were alive without disease, 1 patient had multiple recurrences and 3 died of their disease.
There was an observable rise of incidence of sarcomas on post-COVID era and a more aggressive tumor behavior compared to pre-COVID era. Furthermore, the sarcomas/total malignancies ratio has also increased: during pre-COVID era it was 1,14% while during post- COVID era it was 8,62%.
Conclusions: Besides the evolution in diagnostics, imaging, chemotherapy and surgery, treating craniofacial sarcomas can be really challenging. The prognosis is still poor. More studies and further investigation, with a reliable number of cases, need to be carried out in order to shed light to the overall treatment strategy considering the anatomy and the proximity with vital structures.
KEY WORDS:
Authors:
Hatzopoulos G, Papakosta V, Shihada A, Kardara-Bellou M, Mpalakas A, Lefantzis N, Vasiliou S
Affiliation:
Department of Oral and Maxillofacial Surgery, Attikon Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Doi: 10.54936/haoms242p44
ABSTRACT:
Objectives: TSoft tissue sarcomas of craniofacial area constitute very rare malignancies with histological and clinical diversity. This study aims to present our clinical experience on craniofacial soft tissue sarcomas in pre-COVID and post-COVID era. A literature review is also conducted with emphasis to epidemiology, diagnosis, treatment and prognosis.
Materials and Methods: We reviewed the cases of 7 patients with craniofacial soft tissue sarcomas that were treated in our Clinic the period 2012-2022. We registered the following information: sarcoma type, diagnostic criteria, disease status at presentation, treatment, outcome and differences in pre- and post-covid era.
Results: There were 6 men and 1 woman with a median age of 56. These cases included: 1 scalp pleiomorphic sarcoma, 1 parotid gland liposarcoma, 1 supraorbital myofibroblastic sarcoma, 1 paranasal Malignant Peripheral Nerve Sheath Tumor, 1 paranasal synovial sarcoma, 1 paranasal angiosarcoma and 1 dermatofibrosarcoma. The diagnosis was histological in all cases. Most cases presented with AJCC stage II disease. Treatment was multidisciplinary. Extensive surgical excision was used in all cases, except 1 due to patient’ s denial. Surgical margins were microscopically positive in 3 patients. Induction chemotherapy was used in the case of MPNST and postoperative chemoradiotherapy in the case of dermatofibrosarcoma. At last follow up 3 patients were alive without disease, 1 patient had multiple recurrences and 3 died of their disease.
There was an observable rise of incidence of sarcomas on post-COVID era and a more aggressive tumor behavior compared to pre-COVID era. Furthermore, the sarcomas/total malignancies ratio has also increased: during pre-COVID era it was 1,14% while during post- COVID era it was 8,62%.
Conclusions: Besides the evolution in diagnostics, imaging, chemotherapy and surgery, treating craniofacial sarcomas can be really challenging. The prognosis is still poor. More studies and further investigation, with a reliable number of cases, need to be carried out in order to shed light to the overall treatment strategy considering the anatomy and the proximity with vital structures.
KEY WORDS: