Humans ; Mesenchymoma/surgery* ; Soft Tissue Neoplasms ; Osteomalacia

Endoscopy ; Female ; Humans ; Mesenchymoma ; surgery ; Nose Neoplasms ; surgery ; Young Adult

Objective: To investigate the diagnosis and surgical treatment of sinonasal phosphaturic mesenchymal tumor (PMT). Methods: The medical records of nine patients who had been diagnosed as sinonasal PMT in Department of Otorhinolaryngology Head and Neck Surgery, Shanghai JiaoTong University Affiliated Sixth People's Hospital between January 2015 and May 2020 were collected, including 4 males and 5 females, ranging from 36 to 59 years. The patient's previous history, clinical manifestations, imaging findings, laboratory results, surgical procedure, pathological results and postoperative follow-up data were analyzed by descriptive statistical analysis. Results: All patients presented hypophosphatemia and tumor-induced osteomalacia (TIO) with a disease course of 1 to 19 years. The imaging examination and intraoperative findings identified two cases with peripheral tissue infiltration, two cases with contralateral nasal cavity invasion, and one case with intracranial invasion. Five patients underwent unilateral endoscopic resection while two patients underwent bilateral endoscopic resection, and the remaining two patients underwent unilateral transorbital ethmoid artery ligation plus endoscopic tumor resection and endoscopic combined with transfrontal tumor resection (n=1 each). Expect for one case developed recurrence and intracranial involvement, the other patients achieved clinical remission and no recurrence was observed during the six-month follow-up. Conclusions: The diagnosis of sinonasal PMT needs combination of clinical manifestation, imaging, and pathological findings. Complete surgical excision and long-term postoperative follow-up are imperative.
China ; Female ; Humans ; Hypophosphatemia ; Male ; Mesenchymoma/surgery* ; Neoplasm Recurrence, Local ; Neoplasms, Connective Tissue/surgery* ; Retrospective Studies

Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1). At present, ectomesenchymal cells that migrate from the neural crest are considered to play a pivotal role in tumor origin. All cases had a benign course, although there were three recurrences. Because of the rarity of this tumor and the need for differential diagnostic differentiation from myoepithelioma and pleomorphic adenoma, both oral surgeons and pathologists should be aware of this entity.
Biomarkers, Tumor ; analysis ; Chondroma ; pathology ; surgery ; Diagnosis, Differential ; Female ; Humans ; Immunophenotyping ; Mesenchymoma ; pathology ; surgery ; Middle Aged ; Myoepithelioma ; pathology ; surgery ; Palatal Neoplasms ; pathology ; surgery

Actins ; metabolism ; Adult ; Antigens, Neoplasm ; metabolism ; Diagnosis, Differential ; Epithelioid Cells ; chemistry ; pathology ; Female ; Humans ; Hysterectomy ; Immunohistochemistry ; Melanoma-Specific Antigens ; Mesenchymoma ; metabolism ; pathology ; surgery ; Neoplasm Proteins ; metabolism ; Uterine Neoplasms ; metabolism ; pathology ; surgery

Tumor-induced osteomalacia (TIO), or oncogenic osteomalacia (OOM), is a rare acquired paraneoplastic disease characterized by renal phosphate wasting and hypophosphatemia. Recent evidence shows that tumor-overexpressed fibroblast growth factor 23 (FGF23) is responsible for the hypophosphatemia and osteomalacia. The tumors associated with TIO are usually phosphaturic mesenchymal tumor mixed connective tissue variants (PMTMCT). Surgical removal of the responsible tumors is clinically essential for the treatment of TIO. However, identifying the responsible tumors is often difficult. Here, we report a case of a TIO patient with elevated serum FGF23 levels suffering from bone pain and hypophosphatemia for more than three years. A tumor was finally located in first metacarpal bone by octreotide scintigraphy and she was cured by surgery. After complete excision of the tumor, serum FGF23 levels rapidly decreased, dropping to 54.7% of the preoperative level one hour after surgery and eventually to a little below normal. The patient's serum phosphate level rapidly improved and returned to normal level in four days. Accordingly, her clinical symptoms were greatly improved within one month after surgery. There was no sign of tumor recurrence during an 18-month period of follow-up. According to pathology, the tumor was originally diagnosed as "lomangioma" based upon a biopsy sample, "proliferative giant cell tumor of tendon sheath" based upon sections of tumor, and finally diagnosed as PMTMCT by consultation one year after surgery. In conclusion, although an extremely rare disease, clinicians and pathologists should be aware of the existence of TIO and PMTMCT, respectively.
Bone Neoplasms ; blood ; complications ; diagnostic imaging ; pathology ; surgery ; Female ; Fibroblast Growth Factors ; blood ; Follow-Up Studies ; Humans ; Hypophosphatemia ; blood ; diagnostic imaging ; etiology ; pathology ; surgery ; Mesenchymoma ; blood ; complications ; diagnostic imaging ; pathology ; surgery ; Metacarpal Bones ; Middle Aged ; Neoplasms, Connective Tissue ; blood ; complications ; diagnostic imaging ; pathology ; surgery ; Osteomalacia ; blood ; diagnostic imaging ; etiology ; pathology ; surgery ; Phosphates ; blood ; Radiography

Desmin ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Infant ; Ki-67 Antigen ; metabolism ; Mesenchymoma ; drug therapy ; metabolism ; pathology ; surgery ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Phosphopyruvate Hydratase ; metabolism ; Rhabdomyosarcoma, Embryonal ; drug therapy ; metabolism ; pathology ; surgery ; Teratoma ; metabolism ; pathology ; Vulvar Neoplasms ; drug therapy ; metabolism ; pathology ; surgery

Actins ; metabolism ; Bone Neoplasms ; blood ; complications ; diagnostic imaging ; pathology ; surgery ; Diagnosis, Differential ; Female ; Humans ; Hypophosphatemia ; blood ; etiology ; Ilium ; Mesenchymoma ; blood ; complications ; diagnostic imaging ; pathology ; surgery ; Middle Aged ; Osteomalacia ; blood ; etiology ; Phosphates ; blood ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Tomography, X-Ray Computed

12E7 Antigen ; Aged ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Diagnosis, Differential ; Giant Cell Tumors ; pathology ; Giant Cells ; pathology ; Humans ; Male ; Mesenchymoma ; pathology ; Mucin-1 ; metabolism ; Myositis Ossificans ; pathology ; Osteosarcoma ; metabolism ; pathology ; surgery ; Penile Neoplasms ; metabolism ; pathology ; surgery ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Vimentin ; metabolism