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The American Journal of Surgical... Jun 2021Cutaneous anaplastic large-cell lymphoma (C-ALCL) represents one of the entities within the group of CD30-positive lymphoproliferative disorders of the skin. Most cases...
Spindle-cell (Sarcomatoid) Variant of Cutaneous Anaplastic Large-cell Lymphoma (C-ALCL): An Unusual Mimicker of Cutaneous Malignant Mesenchymal Tumors-A Series of 11 Cases.
Cutaneous anaplastic large-cell lymphoma (C-ALCL) represents one of the entities within the group of CD30-positive lymphoproliferative disorders of the skin. Most cases are ALK-negative, though isolated cases of ALK-positive C-ALCL have also been reported. By definition, the diagnosis of C-ALCL requires the expression of CD30 in >75% of the cells. Histopathologically, C-ALCL shows a dermal-based nodular and circumscribed proliferation of large pleomorphic cells with vesicular nuclei, prominent nucleoli, and eosinophilic cytoplasm, including hallmark cells. Since 1990, isolated case reports of a so-called "sarcomatoid" variant have been published in the literature. Herein, we present a series of 11 cases of spindle (sarcomatoid) C-ALCL, with comprehensive histopathologic, immunophenotypic, and molecular data. Spindle C-ALCL represents a potential mimicker of malignant mesenchymal or hematopoietic tumors in the skin and should always be considered in the differential diagnosis when assessing cutaneous pleomorphic spindle cell neoplasms.
Topics: Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Immunophenotyping; In Situ Hybridization, Fluorescence; Lymphoma, Primary Cutaneous Anaplastic Large Cell; Male; Mesenchymoma; Middle Aged; Predictive Value of Tests; Skin Neoplasms
PubMed: 33234878
DOI: 10.1097/PAS.0000000000001623 -
The Indian Journal of Medical Research Nov 2020
Topics: Humans; Mesenchymoma; Soft Tissue Neoplasms
PubMed: 35345219
DOI: 10.4103/ijmr.IJMR_2371_19 -
Biomolecules Oct 2020Synovial sarcoma (SS) is a malignant mesenchymal soft tissue neoplasm. Despite its name, the cells of origin are not synovial cells, but rather neural, myogenic, or...
Synovial sarcoma (SS) is a malignant mesenchymal soft tissue neoplasm. Despite its name, the cells of origin are not synovial cells, but rather neural, myogenic, or multipotent mesenchymal stem cells have been proposed as possible cells originators. Unlike other sarcomas, an unusual presentation of long-term pain at the tumor site has been documented, but the exact mechanisms have not been fully clarified yet. The transient receptor potential ankyrin 1 (TRPA1) is a nonselective cation channel mainly expressed in primary sensory neurons, where it functions as a pain sensor. TRPA1 have also been described in multiple non-excitable cells, including those derived from neural crest stem cells such as glial cells and, in particular, Schwann cell oligodendrocytes and astrocytes. We evaluated TRPA1 expression in SS. We selected a cohort of 41 SSs, and by immunohistochemistry, we studied TRPA1 expression TRPA1 was found in 92.6% of cases. Triple TRPA1/pS100/SOX10 and TRPA1/SLUG/SNAIL staining strongly supports a neural origin of SS. TRPA1 positivity was also observed in a subset of cases negative with pS100, SOX10 and/or SLUG/SNAIL, and these divergent phenotypes may reflect a process of tumor plasticity and dedifferentiation of neural-derived SSs. Given the functional diversity of TRPA1 and its expression in neuronal and non-neuronal multipotent neural crest stem cells, it remains to be determined whether TRPA1 expression in SSs neoplastic cells plays a role in the molecular mechanism associated with premonitory pain symptoms and tumor progression.
Topics: Adolescent; Adult; Aged; Biomarkers, Tumor; Female; Gene Expression Regulation, Neoplastic; Humans; Male; Mesenchymoma; Middle Aged; Neural Stem Cells; Sarcoma, Synovial; Soft Tissue Neoplasms; TRPA1 Cation Channel; Young Adult
PubMed: 33076385
DOI: 10.3390/biom10101446 -
Ear, Nose, & Throat Journal Jun 2022
Topics: Humans; Mesenchymoma; Paraneoplastic Syndromes; Soft Tissue Neoplasms
PubMed: 32729744
DOI: 10.1177/0145561320940869 -
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 -
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 -
Neurology India 2019Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome of certain mesenchymal tumors which secrete fibroblast growth factor-23 (FGF-23) responsible for... (Review)
Review
Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome of certain mesenchymal tumors which secrete fibroblast growth factor-23 (FGF-23) responsible for causing features of hypophosphatemia and osteomalacia in these patients. Most of them involve the appendicular skeleton and occasionally the craniofacial regions. Involvement of spine is exceedingly rare. Through this paper, the authors present a rare case of a 71-year-old male with TIO due to a lesion in the cervical spine (right C2 lamina) which was proven to be a phosphaturic mesenchymal tumor-mixed connective tissue type on histopathology. This is the fifth reported case of TIO localized to the cervical spine. The patient underwent a hemilaminectomy and gross total resection of the tumor following which he made a gradual but steady recovery and does not have any recurrence 24 months after surgery. The authors not only provide a comprehensive literature review of all 18 spinal cases reported till date but also discuss the management of these patients in light of the published literature.
Topics: Aged; Cervical Vertebrae; Fibroblast Growth Factor-23; Humans; Male; Mesenchymoma; Middle Aged; Neoplasms, Connective Tissue; Osteomalacia; Paraneoplastic Syndromes; Spinal Neoplasms
PubMed: 31744971
DOI: 10.4103/0028-3886.271274 -
BMJ Case Reports Oct 2019Ectomesenchymal chondromyxoid tumour (ECMT) is a rare benign tumour which classically presents in the anterior tongue. This tumour is grossly under-reported due to lack...
Ectomesenchymal chondromyxoid tumour (ECMT) is a rare benign tumour which classically presents in the anterior tongue. This tumour is grossly under-reported due to lack of immunohistochemical staining in many centres. We report a 46-year-old man who presented with mass in the anterior tongue and was diagnosed with ECMT. Further management of this lesion is explained with a review of the literature.
Topics: Chondroma; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Mesenchymoma; Middle Aged; Tongue Neoplasms
PubMed: 31653634
DOI: 10.1136/bcr-2019-231278