Secondary undifferentiated pleomorphic sarcoma of the mandible in a HIV patient who underwent radiotherapy for oral carcinoma

Abstract

Undifferentiated pleomorphic sarcoma (UPS), also defined malignant fibrous histiocytoma (MFH), is one of the most common sarcomas affecting soft tissues in adults, but it can also arise as a primary tumor in bone. UPS is extremely uncommon in the lower jaw and presents very aggressive clinical behavior with high rate of local recurrences and frequent metastases. Secondary UPS are even rarer than primary forms, and they are usually closely associated with local trauma or radiotherapy. This case report aims at illustrating a rare case of secondary UPS of the mandible in an 81-year-old male affected by HIV, maxillary oral squamous cell carcinoma (and mandibular recurrence), and who underwent head and neck radiotherapy. The complete immunohistochemical pattern of the lesion is reported. Due to his medical history and systemic status, the patient has been considered not eligible for further surgical procedures nor radiotherapy. Therefore, a palliative care treatment has been scheduled.

Primary oral leiomyosarcoma of the maxillary bone and sinus: case report and up-to-date review of literature

“…11 Moreover, due to overlapping histological features between different types of head and neck sarcomas, this lesion could be also misclassified for other more common spindle cell tumors of the oral cavity. 3,5,13 For this reason, histological examination associated with immunohistochemical confirmation is mandatory to obtain a definitive diagnosis of oral LMS. 1,3,11,13 Surgical resection with tumour-free margins is widely accepted as the treatment of choice for oral LMS.…”

“…3,5,13 For this reason, histological examination associated with immunohistochemical confirmation is mandatory to obtain a definitive diagnosis of oral LMS. 1,3,11,13 Surgical resection with tumour-free margins is widely accepted as the treatment of choice for oral LMS. 1,3,5,11 In cases where the anatomical location of tumor does not allow radical surgical intervention, radiotherapy and administration of neoadjuvant/adjuvant chemotherapy may improve survival time and decrease or delay the recurrence rate.…”

Giant pleomorphic sarcoma of the tongue base – a cured clinical case report and literature review

“…CT imaging may suggest UPS if a voluminous, multilobed mass with a cervical muscle-like density is present, calcifications in 5–20% of cases, center with low density due to tumor necrosis or the presence of a myxoid material, with heterogeneous loading at the administration of the contrast substance. MRI shows a heterogeneous signal in all sequences, sometimes with areas of intratumorally hemorrhage [ 14 ].…”

Maxillary extrafollicular adenomatoid odontogenic tumor: root resorption and involvement of the maxillary sinus and nasal cavity

“…The differential diagnosis should be made with odontogenic cysts, ameloblastoma, fibro-osseous lesions and tumors of mesenchymal origin [4,5].…”