暂无摘要。

BACKGROUND: Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone.
METHODS: A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up.
CONCLUSIONS: Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.

UNASSIGNED: Endolymphatic sac tumor (ELST) is a rare lesion. It may be sporadically or associated with Von Hippel-Lindau syndrome. Progressive audiovestibular symptoms characterize the typical clinical presentation. Here, we report a unique case of ELST with acute intracranial hypertension (IH) due to tumor compression, successfully treated with an urgent suboccipital decompressive craniectomy (SDC).
UNASSIGNED: A 33-year-old woman previously underwent a biopsy and ventriculoperitoneal shunt. The histopathological finding revealed an ELST. One year later, she developed headache, vomiting, and somnolence due to brainstem compression. An urgent SDC was performed. One month later, preoperative endovascular embolization and partial tumor resection were carried out. After 6 months adjuvant radiotherapy (RT) therapy was administered. She has been under follow-up for 8 years since the last surgical procedure, and the tumor remains stable.
UNASSIGNED: ELST generally has a progressive clinical course. This is a unique case with acute IH due to tumor compression. The tumor\'s high vascularity and the unavailability of endovascular embolization precluded its resection. SDC was an alternative approach. The final treatment included tumor embolization, surgical resection, and RT. No progression was observed for 8 years after the last procedure, and long-term follow-up is warranted.

Esthesioneuroblastoma is a rare malignant tumor developing from the olfactory neuroepithelium. It represents less than 5% of all cancers of the nasal cavity. We are going to report the observation of a patient followed at the regional oncology center of Oujda in Morocco who presented a locally advanced esthesioneuroblastoma. Treatment consisted of surgical resection followed by adjuvant radiotherapy on the tumor bed. Currently, the patient is in good control of his disease.

Metaplastic Breast Cancer (MpBC) is an exceedingly rare entity, accounting for less than 1% of all malignant breast tumours. Predominantly triple-negative, they are notorious for their chemoresistance, high rates of recurrence and decreased disease-free survival (DFS). All this contributes significantly to BC mortality and results in poor prognostic implications. Limited evidence has led to a lacuna of specific treatment guidelines for this entity and hence remains an uncharted territory for clinicians.
We report a case of a 46 year old premenopausal female with left-sided metaplastic triple negative T3N2aM0 BC with mesenchymal differentiation (high grade) whom we treated with neoadjuvant chemotherapy, primary surgery in the form of extreme oncoplasty and adjuvant radiotherapy by Telecobalt machine. Contrary to the expected aggressive course of the disease and poor prognosis of treatment, the patient is presently in remission without progression for over 2 years of follow up.
Limited experience in management of this pathological entity warrants the need for more research on it, with a special focus on targeted therapy. Discussing possibilities of a tailored approach, rather than a one-size-fits-all approach may aid in paving the path for the future of MpBC treatment.

BACKGROUND: Locally advanced rectal cancer (LARC) is commonly managed with neoadjuvant chemoradiation (neoCRT) followed by surgery, though not without complications. The anatomical exposure of the colon and rectum and pelvic radiotherapy poses risk, with rectal perforation and bowel obstruction, though rare, carrying life-threatening potential.
METHODS: This case highlights an exceptionally rare occurrence of concurrent rectal perforation and rectal obstruction in a 77-year-old male with LARC, just two months post neoCRT. Initial symptoms included rectal bleeding, and diagnostic procedures confirmed rectal T1N3adenocarcinoma with no metastasis. Emergency admission, prompted by complete bowel obstruction symptoms, led to discovery of rectal perforation during laparotomy, sealed by the bladder. Pathological analysis attributed the cause to radiation proctitis, reporting complete response to neoCRT with no residual tumor.
CONCLUSIONS: The rarity of both bowel obstruction and perforation as neoCRT complications, particularly in the acute phase of radiation proctitis, is noteworthy in this case. The absence of tumoral cells at the affected sites emphasizes the exceptional nature of this case.
CONCLUSIONS: This case underscores the importance of recognizing acute post neoCRT injuries as potentially life-threatening complications, emphasizing the need for heightened awareness and consideration in clinical management.

Intracranial primary chondrosarcomas are rare, accounting for <0.15% of all intracranial tumors, but exhibit a high risk of recurrence. Due to the rarity of this condition, it has proven difficult to establish efficacy-based treatment guidelines. The present study details a case of clivus chondrosarcoma exhibiting no recurrence following surgical resection using an endoscopic transsphenoidal approach and postoperative adjuvant radiotherapy. A 41-year-old female presented with primary symptoms of left eye esotropia, scotoma of the left nasal visual field and double vision. Preoperative cranial magnetic resonance imaging revealed a lesion on the clivus, which was initially diagnosed as chordoma. However, clivus chondrosarcoma was ultimately diagnosed based on intraoperative findings and postoperative histopathology. The tumor was totally resected and 25 doses of adjuvant radiotherapy with planning gross tumor volume (60 Gy) and planning clinical target volume (50 Gy) were administered for 5 weeks. The patient was discharged at 12 days post-surgery with no obvious postoperative complications. Over the 28-month follow-up period, there was no evidence of recurrence, which may be due to the successful use of combined gross total resection and adjuvant radiotherapy. Therefore, surgical resection using an endoscopic transsphenoidal approach and postoperative adjuvant radiotherapy is an effective method for treating intracranial clivus chondrosarcoma.

暂无摘要。

BACKGROUND: TGCT (tenosynovial giant cell tumor) is a mono-articular proliferative condition that begins in the synovial membranes. Diffuse TGCT (DTGCT) has more prominent symptoms with high risks of recurrency.
METHODS: In this case, we presented a 32-year-old male with recurrent case of DTGCT. The patient has been treated with arthroscopic synovectomy with the recurrence of disease. The patient was finally treated by open synovectomy combined with arthroscopy followed by adjuvant radiotherapy.
CONCLUSIONS: The aim of this case is to report the success of open synovectomy combined with arthroscopy followed by 31 cycle of adjuvant radiotherapy in the recurrent case of DTGCT.
CONCLUSIONS: Open synovectomy combined with arthroscopic synovectomy followed by adjuvant RT is a promising treatment modality in patient with DTGCT.

BACKGROUND: Budd-Chiari syndrome (BCS) is a rare vascular disorder of the liver, and acute and secondary BCS is even rarer.
METHODS: A 62-year-old man with perihilar cholangiocarcinoma of Bismuth type IIIa underwent right hemi-hepatectomy with caudate lobectomy and pancreatoduodenectomy. Adjuvant chemoradiotherapy was performed due to a positive hepatic ductal margin. Subsequently, the disease passed without recurrence. The patient visited for acute onset abdominal pain at the 32nd postoperative month. Multidetector-row computed tomography (MDCT) showed stenosis of the left hepatic vein (LHV) root, which was the irradiated field, and thrombotic occlusion of the LHV. The patient was diagnosed with acute BCS caused by adjuvant radiotherapy. Although anticoagulation therapy was performed, the patient complained of sudden upper abdominal pain again. MDCT showed an enlarged LHV thrombus and hepatomegaly. The patient was diagnosed with exacerbated acute BCS, and stenting for the stenotic LHV root was performed with a bare stent. Although stenting for the LHV root was very effective, restenosis occurred twice due to thrombus in the existing stent, so re-stenting was performed twice. The subsequent clinical course was acceptable without recurrence or restenosis of the LHV root as of 6 months after the last stenting using a stent graft.
CONCLUSIONS: Although no case of BCS caused by radiotherapy has yet been reported, the present case showed that late side effect of radiotherapy can cause hepatic vein stenosis and secondary BCS.