Clear cell odontogenic carcinoma (CCOC), a very rare neoplasm located mostly in the mandible, has been regarded as a benign tumor. However, due to the accumulation of case reports, CCOC has been reclassified as a malignant entity by the World Health Organization. Patients with CCOC present with regional swelling and periodontal indications with variable pain, often remaining misdiagnosed for a long period. CCOC has slow growth but aggressive behavior, requiring radical resection. Histologic analysis revealed the monophasic, biphasic, and ameloblastic types of CCOC with clear cells and a mixed combination of polygonal and palisading cells. At the molecular level, CCOC shows the expression of cytokeratin and epithelial membrane antigen, along with markers that assign CCOC to the sarcoma family. At the genetic level, Ewing sarcoma breakpoint region 1-activating transcription factor 1 fusion is regarded as the key feature for identification. Nevertheless, the scarcity of cases and dependence on histological data delay the development of an efficient therapy. Regarding the high recurrence rate and the potential of distant metastasis, further characterization of CCOC is necessary for an early and accurate diagnosis.
Ameloblasts ; Diagnosis ; Humans ; Keratins ; Mandible ; Mucin-1 ; Neoplasm Metastasis ; Odontogenic Tumors ; Recurrence ; Sarcoma ; Sarcoma, Ewing ; Transcription Factors ; World Health Organization

PURPOSE: Extraosseous Ewing's sarcoma (EOE) of the rectum is extremely rare: only three cases have been reported in the literature and none of these reports described their imaging findings in detail. Herein, we describe the tumor imaging and pathological features in detail. MATERIALS AND METHODS: We report a case of rectal EOE in a 72-year-old female who received local excision and was provisionally diagnosed with a rectal submucosal spindle cell tumor. We used immunohistochemistry, histopathology, and fluorescence in situ hybridization to characterize the tumor and provide a definitive diagnosis of EOE. RESULTS: MRI revealed a well-demarcated submucosal tumor with heterogeneous enhancement and hemorrhagic foci in rectum. EOE was diagnosed by positive staining of tumor cells for CD99 and Fli-1 by immunohistochemistry and the presence of the EWSR1 gene translocation by fluorescence in situ hybridization. Although the patient underwent radiation treatment and surgery, the tumor recurred after 4 months as revealed by computed tomography and magnetic resonance imaging. CONCLUSION: Rectal EOE may present as a rectal submucosal tumor. The understanding of imaging and histological characteristics of this tumor are critical for accurate diagnosis and appropriate aggressive treatment.
Aged ; Diagnosis ; Female ; Fluorescence ; Humans ; Immunohistochemistry ; In Situ Hybridization ; Magnetic Resonance Imaging ; Rectum ; Sarcoma, Ewing*

Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are a group of malignant tumors with varying degrees of neuroectodermal differentiation. Although it may develop in any organs, ES/PNET originating from small intestine is exceedingly rare. We experienced a 9-year-old girl presenting with abdominal pain, melena, and iron deficiency anemia. Imaging work-up showed multiple masses in the small bowel and omentum with disseminated peritoneal seeding nodules, indicating lymphoma as the most likely diagnosis. Pathological reports from explorative diagnostic laparoscopic biopsy showed tumors comprising small round cells with CD99 expression and EWS-FLI1 translocation leading to the diagnosis of ES/PNET. Tumor burden decreased gradually during five consecutive cycles of systemic chemotherapy. The patient received segmental resection of jejunum, followed by adjuvant chemotherapy. This is the first pediatric case of ES/PNET found in small intestine in Korea.
Abdominal Pain ; Anemia, Iron-Deficiency ; Biopsy ; Chemotherapy, Adjuvant ; Child ; Diagnosis ; Drug Therapy ; Female ; Humans ; Intestine, Small ; Jejunum ; Korea ; Lymphoma ; Melena ; Neural Plate ; Neuroectodermal Tumors ; Neuroectodermal Tumors, Primitive ; Omentum ; Pediatrics ; Sarcoma, Ewing ; Tumor Burden

Thoracic radiculopathy represents an uncommon spinal disorder that is frequently overlooked in the evaluation of thoracic, or abdominal pain syndrome. The clinical representation of this uncommon disorder is often atypical. With many differential diagnoses to consider, it is not surprising that the cause of thoracic radiculopathy is often not discovered for months, or years, after the symptoms arise. We report two rare cases of thoracic radiculopathy; one case was caused by extraskeletal Ewing sarcoma (EES) along the thoracic paraspinal area, and the other by foraminal stenosis, due to a bony spur of the thoracic vertebra. As such, thoracic radiculopathy should be considered in the diagnosis of patients with thoracic and abdominal pain, especially if initial diagnostic studies are inconclusive.
Abdominal Pain ; Constriction, Pathologic ; Diagnosis ; Diagnosis, Differential ; Humans ; Radiculopathy* ; Sarcoma, Ewing ; Spine

Thoracic radiculopathy represents an uncommon spinal disorder that is frequently overlooked in the evaluation of thoracic, or abdominal pain syndrome. The clinical representation of this uncommon disorder is often atypical. With many differential diagnoses to consider, it is not surprising that the cause of thoracic radiculopathy is often not discovered for months, or years, after the symptoms arise. We report two rare cases of thoracic radiculopathy; one case was caused by extraskeletal Ewing sarcoma (EES) along the thoracic paraspinal area, and the other by foraminal stenosis, due to a bony spur of the thoracic vertebra. As such, thoracic radiculopathy should be considered in the diagnosis of patients with thoracic and abdominal pain, especially if initial diagnostic studies are inconclusive.
Abdominal Pain ; Constriction, Pathologic ; Diagnosis ; Diagnosis, Differential ; Humans ; Radiculopathy* ; Sarcoma, Ewing ; Spine

PURPOSE: It is generally accepted that bony reconstruction after type III (pubic) internal pelvectomy is not necessary. However, technical problems in type III resection, functional outcome according to the extent of resection, and the usefulness of synthetic material to decrease the risk of hernia has not been well addressed. MATERIALS AND METHODS: Fifteen patients who underwent type III internal pelvectomy were extracted and the pathologic diagnosis, Enneking's stage, location of tumor and size, operation time, amount of transfusion, surgical margin, local recurrence, distant metastasis, and functional outcomes were analyzed according to the extent of resection. RESULTS: Pathologic diagnosis was chondrosarcoma in 9, Ewing's sarcoma in 3, metastatic carcinoma in 2, and osteosarcoma in 1 patient. There were 4 patients with local recurrence and one with concomitant lung metastasis. Average Musculoskeletal Tumor Society functional score was 26.7. According to the extent of resection, functional score of 7 cases with unilateral both rami (6) or ischium (1) resection was 26, 4 cases with unilateral both rami and partial contralateral pubic ramus resection was 25, and 4 cases with unilateral both rami including ischium was 24. Two patients had tumor related complication. One patient with a huge intrapelvic tumor aroused at the symphysis pubis showed urethral invasion at presentation, therefore, urethral resection and permanent suprapubic cystostomy was inevitable. The other patient with bilateral pubic ramus involvement by tumor showed caudal displacement of the uterus after pregnancy (4 years after primary resection). She underwent Caesearian section for delivery. CONCLUSION: Regardless of the extent of pubic bone resection, functional outcome was similar. The risk of abdominal or pelvic organ hernia was minimal even without the use of artificial material for soft tissue reconstruction; however, when the extent of resection crosses the symphysis pubis, selective application of an additional procedure to reinforce the pelvic floor may be considered.
Chondrosarcoma ; Cystostomy ; Diagnosis ; Hemipelvectomy* ; Hernia ; Humans ; Ischium ; Lung ; Neoplasm Metastasis ; Osteosarcoma ; Pelvic Floor ; Pelvic Neoplasms ; Pelvis* ; Pregnancy ; Pubic Bone ; Recurrence ; Sarcoma, Ewing ; Treatment Outcome ; Uterus

Clear-cell sarcoma (CCS) is a rare soft tissue sarcoma that usually develops in the lower extremities of young adults. CCS of the gastrointestinal tract is extremely rare. We report here the first case of CCS of the stomach in Korea. A 28-year-old female developed a large tumor of the stomach that was initially considered Ewing sarcoma, based on the results of a needle biopsy. She had paraneoplastic syndrome; fever, anemia, and hyperglycemia. Follow-up abdominal computed tomography after four-cycles of chemotherapy with vincristine-doxorubicin-cyclophosphamide or ifosfamide-etoposide showed no tumor shrinkage and revealed a fistula between the tumor and the gastric lumen. The infected tumor prompted debulking surgery, which resulted in improvement of her symptoms. The pathologic findings were consistent with CCS, and fluorescence in situ hybridization for EWS gene rearrangement was positive, confirming the diagnosis. This case highlights the importance of clinical suspicion for CCS in chemotherapy-refractory Ewing sarcoma.
Adult ; Anemia ; Biopsy, Needle ; Diagnosis ; Drug Therapy ; Female ; Fever ; Fistula ; Fluorescence ; Follow-Up Studies ; Gastrointestinal Tract ; Gene Rearrangement ; Humans ; Hyperglycemia ; In Situ Hybridization ; Korea ; Lower Extremity ; Paraneoplastic Syndromes ; Sarcoma* ; Sarcoma, Clear Cell ; Sarcoma, Ewing ; Stomach* ; Young Adult

Primitive neuroectodermal tumor (PNET), which belongs to the Ewing's sarcoma (ES) family of tumors, is mainly seen in children and young adults. PNETs are extremely rare in the maxilla. Here, we report a case of PNET of the left maxillary sinus in an elderly male. Magnetic resonance imaging (MRI) revealed a slightly enhanced solid mass occupying the left maxillary sinus and infiltrating into the retroantral space. A partial maxillectomy was performed. Despite postoperative chemotherapy, follow-up computed tomography (CT) and MRI revealed a nodal metastasis in the submandibular space. Neck dissection was performed. However, the patient died 10 months after the second surgery because of distant metastasis to the liver. MRI and CT were particularly useful in detecting the extent of the tumor, recurrence, and metastasis. Further, a literature review of the previously reported PNET cases of the maxilla was carried out. In this paper, we also discuss the current approach for the diagnosis and management of these tumors.
Aged* ; Child ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Maxilla ; Maxillary Sinus* ; Neck Dissection ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive* ; Recurrence ; Sarcoma, Ewing ; Young Adult

Adolescent ; Female ; Humans ; Ovarian Neoplasms ; diagnosis ; Sarcoma, Ewing ; diagnosis

Adamantinoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Adult ; Diagnosis, Differential ; Female ; Femur ; Follow-Up Studies ; Humans ; Humerus ; Ilium ; Keratins ; metabolism ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Retrospective Studies ; Sarcoma, Ewing ; pathology ; Sarcoma, Synovial ; pathology ; Tibia ; Tomography, X-Ray Computed ; Young Adult