Nasal Chondromesenchymal Hamartoma in Children

2001 ◽  
Vol 125 (3) ◽  
pp. 400-403 ◽  
Author(s):  
Chuen Hsueh ◽  
Swei Hsueh ◽  
Frank Gonzalez-Crussi ◽  
Ta-jen Lee ◽  
Jen-liang Su

Abstract Hamartoma in the nasal cavity of children is especially rare. Most documented cases occurred in infants, with characteristic histologic features of a mixture of various mesenchymal tissues. McDermott et al designated it nasal chondromesenchymal hamartoma in 1998, and it has since been considered a distinct clinicopathological entity. We report 2 such examples in a full-term male newborn and a 9-month-old boy, respectively. Histologically, both cases were characterized by a mixture of various mesenchymal elements, including spindle cells, collagen fibers, and irregular islands of osseous and chondroid tissue. Immunohistochemical study showed positivity to vimentin and S100 protein. Ultrastructural examination of case 1 demonstrated fibroblastic and myofibroblastic differentiation in tumor cells. There were 11 cases of nasal chondromesenchymal hamartoma in children published to date. The tumor has a benign biological behavior, and complete resection is the treatment of choice. It is apt to be misdiagnosed because of overlapping histologic features shared with a number of benign and malignant soft tissue tumors. Awareness of this entity is essential for correct diagnosis and adequate therapy.

2020 ◽  
pp. 106689692093812
Author(s):  
Oliver Foot ◽  
Magnus Hallin ◽  
Robin L. Jones ◽  
Vaiyapuri P. Sumathi ◽  
Khin Thway

EWSR1-SMAD3-positive fibroblastic tumor is a recently characterized neoplasm with distinct clinicopathologic features and recurrent EWSR1-SMAD3 gene fusion. ESFT typically presents as a small, painless tumor in extremity subcutaneous tissues. Their behavior is benign, although they are prone to local recurrence. They typically comprise two components: intersecting fascicles of overlapping, uniform plump spindle cells, and less cellular hyalinized areas containing stippled calcifications. Immunohistochemically, the cells consistently show diffuse ERG nuclear expression, while other markers are negative. The morphology of this neoplasm can lead to histologic confusion with both benign and malignant soft tissue tumors, including monophasic synovial sarcoma, malignant peripheral nerve sheath tumor, and spindle cell sarcoma, not otherwise specified. Correct identification of ESFT is critical, most importantly to avoid unnecessary overtreatment as sarcoma.


Author(s):  
Brandon K. K. Fields ◽  
Natalie L. Demirjian ◽  
Darryl H. Hwang ◽  
Bino A. Varghese ◽  
Steven Y. Cen ◽  
...  

2012 ◽  
Vol 02 (01) ◽  
pp. 45-47
Author(s):  
Shetty K. Padma ◽  
Harish S. Permi ◽  
C.N. Patil ◽  
Michelle Mathias

AbstractSarcomatoid carcinoma occurring in the small intestine is very rare. They can be monophasic or biphasic. We report a rare case of monophasic Sarcomatoid carcinoma of the small intestine in a 60 year old male patient. The tumor was an ulceronodular mass involving the ileum circumferentially. The tumor infiltrated the full thickness of the intestinal wall and the serosa of an adjacent loop of ileum. Microscopically, the tumor was composed of sheets of malignant spindle cells. The carcinomatous nature of the tumor was evident only after Immunohistochemistry. The diagnosis of sarcomatoid carcinoma should be considered in the differential diagnosis of malignant spindle cell tumor of small intestine and immunohistochemical stains are required for the correct diagnosis.


Author(s):  
Hamidreza Fattahian ◽  
Pejman Mortazavi ◽  
Hamidreza Moosavian ◽  
Hamid Mohyeddin ◽  
Roozbeh Moridpour

Accurate diagnostic approaches to differentiate peripheral nerve sheet tumours from others have not been firmly established. The aim of this case report was to diagnose neurofibroma using a combination of diagnostic imaging, histopathology and immunohistochemistry, which were applied to a canine neurofibroma arising in the left mandible. The tumour was surgically excised and examined histologically. Round or spindle cells, with elongated, dense and homogenous chromatin and pale cytoplasm typical of Schwann cells in an abundant fibromyxomatous stroma, with ruby collagen fibres were seen. Immunohistochemistry demonstrated that S-100 and vimentin were more than 70% positive. Neurofibroma may therefore be recognisable using markers such as S-100 and vimentin.


2009 ◽  
Vol 2009 ◽  
pp. 1-6 ◽  
Author(s):  
Kayo Kuyama ◽  
Sisilia Fusi Fifita ◽  
Masamichi Komiya ◽  
Yan Sun ◽  
Yoshiaki Akimoto ◽  
...  

The purpose of this study was to determine the clinicopathological and immunohistochemical features of lipoma/fibrolipoma with rare occasions as osseous and/or chondroid differentiation in the oral cavity. Two cases of the tumors, who presented with a painless, relatively hard mass on the oral mucosa, were studied. These were consisted of a well-circumscribed mass of fatty tissue with chondroid and significant fibrous component intermixed with the lobules of fat cells with chondroid and woven bone component, respectively. Immunohistochemical study revealed that peripheral spindle cells around chondroid tissue stained diffusely for S-100 & and Sox-9, though peripheral spindle cells around osteoid tissue only stained for RUNX-2. According to review of the literature, lipoma/fibrolipoma with osseous and/or chondroid differentiation was 18 cases. Also fibrolipoma with osseous and chondroid differentiation is the first to be reported here. These results indicated that the cartilage/bone is produced by differentiation of undifferentiated mesenchymal cells of stroma.


2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Géraldine Pairet ◽  
Gaëlle Tilman ◽  
Rafaël Sciot ◽  
Thomas Schubert ◽  
Vasiliki Perlepe ◽  
...  

We report a case of multiple myoepithelioma with synchronous bone and soft tissue tumors, associated with a new genomic alteration of the LPP locus. The lesions occurred in the foot by presenting one lump in the plantar soft tissue, and three lesions were detected in the calcaneus and in the navicular bone. All tumors showed the double immunophenotype of epithelial markers and S100 protein expression. No rearrangement of the EWSR1 and FUS loci was detected as reported in myoepitheliomas. However, molecular karyotyping detected an unbalanced rearrangement of the LPP locus, not involving the HMGA2 locus, which is the most frequent translocation partner observed in benign mesenchymal tumors such as lipomas (of soft tissue as well as parosteal) and pulmonary chondroid hamartoma.


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