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Journal of Orthopaedic Science :... Sep 2021Phosphaturic mesenchymal tumors primarily cause tumor-induced osteomalacia, a rare paraneoplastic syndrome, and half occur in soft tissues. There are few reports about...
BACKGROUND
Phosphaturic mesenchymal tumors primarily cause tumor-induced osteomalacia, a rare paraneoplastic syndrome, and half occur in soft tissues. There are few reports about the surgical margins of these tumors. This study aimed to clarify the optimal surgical margin for phosphaturic mesenchymal tumors by analyzing radiological and histopathological features.
METHODS
This study included eight cases, seven primary and one recurrent, of tumor-induced osteomalacia caused by soft-tissue phosphaturic mesenchymal tumors that were surgically treated between January 2000 and January 2019. We evaluated the radiological and histopathological features of all tumors and investigated the correlation of these features, the surgical margin, and recurrence of hypophosphatemia.
RESULTS
The tumors were located in superficial (n = 5) and deep (n = 3) tissues. Six of the eight tumors had a clear boundary, but five had an irregular margin. Three tumors had a hypointense rim on T2-weighted images, indicating fibrous tumor encapsulation. Histopathological analysis revealed infiltrative growth in six of the eight tumors, which correlated with an irregular margin seen on imaging. Although there was no recurrence in patients treated with an intended wide margin >1 cm, one of the three patients treated with marginal tumor resection experienced a recurrence of hypophosphatemia, with histopathological analysis showing infiltration of subcutaneous fat. In contrast, two tumors with clear boundaries, regular margins, and fibrous capsule seen on imaging, had no infiltrative growth and were cured by marginal resection. In one recurrent case, tumor infiltration was observed in the previous surgical scar, which was not detected on preoperative imaging.
CONCLUSIONS
Soft-tissue phosphaturic mesenchymal tumors with an irregular boundary seen on imaging tend to be infiltrative, especially into subcutaneous fat, and should be treated by at least a 1-cm wide margin resection. Tumors with a fibrous capsule with clear and regular margins are cured by marginal margin resection. These findings could inform surgeons' decisions regarding the resection of soft-tissue phosphaturic mesenchymal tumors.
Topics: Humans; Margins of Excision; Mesenchymoma; Neoplasm Recurrence, Local; Neoplasms, Connective Tissue; Soft Tissue Neoplasms
PubMed: 32798110
DOI: 10.1016/j.jos.2020.07.006 -
Ear, Nose, & Throat Journal Jun 2022
Topics: Humans; Mesenchymoma; Paraneoplastic Syndromes; Soft Tissue Neoplasms
PubMed: 32729744
DOI: 10.1177/0145561320940869 -
The Journal of Foot and Ankle Surgery :... 2020Tumor-induced osteomalacia, a rare and intriguing paraneoplastic syndrome that is usually caused by a phosphaturic mesenchymal tumor, leads to severe pain and...
Tumor-induced osteomalacia, a rare and intriguing paraneoplastic syndrome that is usually caused by a phosphaturic mesenchymal tumor, leads to severe pain and hypophosphatemia. However, during clinical practice, most patients suffer from significant delay of diagnosis and treatment because the symptoms are similar to those of some very common diseases, such as osteoporosis and osteoarthritis. Moreover, physical complaints from postmenopausal women usually exacerbate the possibility of such delays. We describe a case of a postmenopausal woman with crippling bone pain and weakness, who had been diagnosed with a case of simple osteoporosis and osteoarthritis for 3 years, even with fine-needle aspiration biopsy of the offending phosphaturic mesenchymal tumor. After surgical removal of the 2 × 3-cm tumor in her sole, we observed immediate relief of her systemic symptoms, with visual analogue scale improvement from 5 of 10 preoperatively to 2 of 10 5 days after surgery. There were no signs of recurrence during 2-year follow-up. This case highlights the significance of thorough history-taking as a fundamental tool for diagnosis even in the era of advanced technology, and that the awareness of tumor-induced osteomalacia should be raised. Otherwise, such a small localized soft tissue mass would seldom be associated with the severe systemic symptoms.
Topics: Female; Humans; Hypophosphatemia; Mesenchymoma; Neoplasm Recurrence, Local; Neoplasms, Connective Tissue; Osteomalacia; Paraneoplastic Syndromes; Postmenopause
PubMed: 32622675
DOI: 10.1053/j.jfas.2020.04.012 -
The New England Journal of Medicine Jun 2020
Topics: Female; Foot; Foot Diseases; Fractures, Bone; Humans; Hypocalcemia; Hypophosphatemia; Magnetic Resonance Imaging; Mesenchymoma; Middle Aged; Osteomalacia; Paraneoplastic Syndromes; Phosphates; Positron Emission Tomography Computed Tomography
PubMed: 32492308
DOI: 10.1056/NEJMcps1913599 -
The New England Journal of Medicine May 2020
Topics: Bone Diseases, Metabolic; Calcitriol; Female; Foot; Foot Diseases; Fractures, Bone; Humans; Hypocalcemia; Hypophosphatemia; Magnetic Resonance Imaging; Mesenchymoma; Middle Aged; Osteomalacia; Paraneoplastic Syndromes; Phosphates; Positron Emission Tomography Computed Tomography
PubMed: 32459927
DOI: 10.1056/NEJMimc1914302 -
Head and Neck Pathology Mar 2021Ectomesenchymal chondromyxoid tumor is a rare neoplasm of uncertain histogenesis that typically occurs in the anterior dorsal tongue. Recent reports in the literature...
Ectomesenchymal chondromyxoid tumor is a rare neoplasm of uncertain histogenesis that typically occurs in the anterior dorsal tongue. Recent reports in the literature have described rare examples of gingival, palatal and tonsillar lesions. Histologically, ectomesenchymal chondromyxoid tumors are typically well-circumscribed, lacking overtly aggressive features. Herein we report a tumor arising in the right mandible that is morphologically and molecularly consistent with ectomesenchymal chondromyxoid tumor. This case furthers awareness of the extra-glossal distribution of this neoplasm; moreover, it suggests that a subset of these tumors have the potential for locally aggressive behaviour.
Topics: Adult; Female; Humans; Mandibular Neoplasms; Mesenchymoma; Myoepithelioma
PubMed: 32372271
DOI: 10.1007/s12105-020-01169-5 -
International Journal of Oral and... Oct 2020Ectomesenchymal chondromyxoid tumour (ECT) is an extremely rare intraoral mesenchymal tumour. Most of these tumours have been identified on the anterior aspect of the...
Ectomesenchymal chondromyxoid tumour (ECT) is an extremely rare intraoral mesenchymal tumour. Most of these tumours have been identified on the anterior aspect of the dorsal surface of the tongue. ECT is difficult to diagnose because of its rarity. We report a case of ECT arising on the lateral border of the tongue in a 67-year-old woman. The tumour, measuring 20 × 10 mm in size, was surgically removed. Histopathologically, the tumour was composed of small polygonal cells arranged in sheets, with a myxoid or hyalinized stroma. The tumour boundary was clear; however, the tumour showed a multinodular structure expanding along the tongue surface without obvious capsule. Careful examination revealed the tumour nodule to be spreading in a skip lesion-like fashion away from the main part of the tumour in the striated muscle layer. Although there was no evidence of recurrence at 18 months after the surgery, our observations suggest that surgery for ECT resection with a safety margin is more appropriate than enucleation.
Topics: Aged; Female; Humans; Mesenchymoma; Myoepithelioma; Neoplasm Recurrence, Local; Tongue; Tongue Neoplasms
PubMed: 32371177
DOI: 10.1016/j.ijom.2020.04.010 -
Magyar Onkologia Mar 2020Based on our current knowledge, 5-10% of all malignancies are part of hereditary cancer syndromes. Although the increasing diagnostic role of molecular genetic testing... (Review)
Review
Based on our current knowledge, 5-10% of all malignancies are part of hereditary cancer syndromes. Although the increasing diagnostic role of molecular genetic testing makes us able to recognize more hereditary cancer patients, the careful exploration of family and clinical history by physicians is still the most important step for the diagnosis. In our review we deal with mesenchymal tumours associated with hereditary syndromes. Sarcomas comprise only 1% of all malignancies, but they often associate with familiar diseases so they can serve as an indicator of these syndromes. The diagnosis of hereditary cancer predisposition syndromes is essential to ensure appropriate therapy and follow-up for our patients.
Topics: Genetic Predisposition to Disease; Genetic Testing; Humans; Mesenchymoma; Neoplastic Syndromes, Hereditary; Sarcoma
PubMed: 32181763
DOI: No ID Found -
Taiwanese Journal of Obstetrics &... Mar 2020To evaluate the risk of encountering unexpected uterine smooth muscle tumors of uncertain malignant potential (STUMPs) or sarcomas during surgical treatment of...
OBJECTIVE
To evaluate the risk of encountering unexpected uterine smooth muscle tumors of uncertain malignant potential (STUMPs) or sarcomas during surgical treatment of mesenchymal tumors of the uterus using morcellation.
MATERIAL AND METHODS
Data were collected retrospectively from subjects who were pathologically diagnosed with uterine leiomyoma or its variants, STUMP or other premalignant mesenchymal tumors of uterus, or sarcoma during surgical treatment between July 2014 and June 2017.
RESULTS
A total of 3785 women were investigated; 2824 laparoscopic procedures (74.6%) were performed, and an electronic power morcellator was used in 1636 patients (43.2%). Sixteen women (0.42%) were diagnosed with STUMP and 14 (0.37%) were diagnosed with uterine sarcoma. The incidence rate of unexpected STUMP or uterine sarcoma was 0.61% (23 of 3785 women); unexpected STUMP in 13 (0.34%), and unexpected sarcoma was in 10 (0.26%). Moreover, the unexpected leiomyosarcoma rate was 0.08% (3 in 3785). The rate of unintended morcellation of STUMPs was relatively high at 0.26% (10 in 3785), however, that for uterine sarcomas was 0.05% (2 in 3785).
CONCLUSION
The risks of unintended morcellation were very low for sarcomas and STUMPs, although the risk of the latter was approximately 5-fold that of the former. To reduce the unintended dissemination of tumors, patients suspected of having malignancies should be provided adequate information regarding their treatment options as well as their associated risks. Meanwhile, improved preoperative screening methods for STUMP and sarcoma should be established.
Topics: Adult; Female; Humans; Incidence; Leiomyosarcoma; Mesenchymoma; Middle Aged; Morcellation; Postoperative Complications; Republic of Korea; Retrospective Studies; Sarcoma; Smooth Muscle Tumor; Uterine Neoplasms
PubMed: 32127150
DOI: 10.1016/j.tjog.2020.01.017 -
Journal of Toxicologic Pathology Jan 2020A rare spontaneous hepatic leiomyosarcoma with osteosarcomatous differentiation was observed in a female beagle dog and its morphological and immunohistochemical...
A rare spontaneous hepatic leiomyosarcoma with osteosarcomatous differentiation was observed in a female beagle dog and its morphological and immunohistochemical characteristics were examined. Upon necropsy, an endoceliac mass originating from the liver was detected, which was composed of hematoid fluid-filled cysts and white to grayish solid tissue. There were no macroscopic findings in other organ systems. Histopathologically, the hepatic mass consisted of two different mesenchymal components. One form was spindle cells arranged in interlacing fascicles immunohistochemically positive for smooth muscle actin (SMA) and smoothelin, indicating leiomyosarcomatous differentiation. The other form was composed of short spindle cells positive for S-100 and was producing various amounts of eosinophilic osteoid and trabecula-like matrices positive for osteocalcin, indicating osteosarcomatous differentiation. In addition, invasive growth in the hepatic parenchyma and cell atypia were observed. Based on these findings, the mass was diagnosed as hepatic leiomyosarcoma with osteosarcomatous differentiation (malignant mesenchymoma), which might be derived from undifferentiated mesenchymal cells.
PubMed: 32051664
DOI: 10.1293/tox.2019-0065