Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity
Abstract Background As a new entity included in the 4th edition of the WHO classification of tumours of soft tissue and bone in 2013, hybrid peripheral nerve sheath tumours are benign composite neoplasms that demonstrate features of more than one type of nerve sheath tumour, with a wide age distribu...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2019-06-01
|
| Series: | Diagnostic Pathology |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13000-019-0829-x |
| _version_ | 1818144029863313408 |
|---|---|
| author | Yuejiao Lang Dawei Liu Pei Xiang Jilin Wang Yang Li |
| author_facet | Yuejiao Lang Dawei Liu Pei Xiang Jilin Wang Yang Li |
| author_sort | Yuejiao Lang |
| collection | DOAJ |
| description | Abstract Background As a new entity included in the 4th edition of the WHO classification of tumours of soft tissue and bone in 2013, hybrid peripheral nerve sheath tumours are benign composite neoplasms that demonstrate features of more than one type of nerve sheath tumour, with a wide age distribution and a predilection for superficial location. Those involving deep sites are relatively rare. To the best of our knowledge, only one case of primary intraosseous hybrid peripheral nerve sheath tumours has been documented. In this article, we report another case of hybrid peripheral nerve sheath tumours occurring in bone with different clinical, radiological and pathological features from those in the previously reported cases. Case presentation A 28-year-old female presented with a painful nodule in the right tibia. Radiological examination revealed an oval eccentric osteolytic lesion in the proximal tibia. Histologically, the circumscribed but unencapsulated lesion demonstrated biphasic cellular differentiation. Bland, small epithelioid cells arranged in clusters in the myxoid or collagenous stroma and inconspicuous spindle cells scattered in the hypercellular areas were suggested to originate from Schwann cells according to the detection of S100. Both the elongated spindle cells with thin, wavy nuclei and the spindle cells in fascicular or storiform pattern in hypercellular areas showed a positive immunoreaction for epithelial membrane antigen, indicating perineurial differentiation. Based on histological and immunochemical examinations, the patient was diagnosed with hybrid epithelioid schwannoma/perineurioma. The lesion was resected and has not recurred for 8 months since resection. Conclusion The present case is the second primary intraosseous hybrid peripheral nerve sheath tumour to be reported. This is also the first reported intraosseous tumour composed of epithelioid schwannoma and perineurioma with hypercellularity, indicating diverse involvement sites and a wide range of histological features among hybrid peripheral nerve sheath tumours. Awareness of such diversity is critical for accurate diagnoses. The morphological overlap with other spindle and epithelioid cell neoplasms, especially pure peripheral nerve sheath tumours, requires that immunochemical and molecular examinations be used as objective tools to provide the necessary information for a differential diagnosis. |
| first_indexed | 2024-12-11T11:41:04Z |
| format | Article |
| id | doaj.art-508c25ed2a144503b744bc1078affb9e |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-12-11T11:41:04Z |
| publishDate | 2019-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-508c25ed2a144503b744bc1078affb9e2022-12-22T01:08:36ZengBMCDiagnostic Pathology1746-15962019-06-011411810.1186/s13000-019-0829-xPrimary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularityYuejiao Lang0Dawei Liu1Pei Xiang2Jilin Wang3Yang Li4Department of Pathology, The First Affiliated Hospital, Sun Yat-sen UniversityDepartment of Pathology, The First Affiliated Hospital, Sun Yat-sen UniversityDepartment of Medical Imaging, The First Affiliated Hospital, Sun Yat-sen UniversityDepartment of Pathology, Central People’s Hospital of ZhanjiangDepartment of Pathology, The First Affiliated Hospital, Sun Yat-sen UniversityAbstract Background As a new entity included in the 4th edition of the WHO classification of tumours of soft tissue and bone in 2013, hybrid peripheral nerve sheath tumours are benign composite neoplasms that demonstrate features of more than one type of nerve sheath tumour, with a wide age distribution and a predilection for superficial location. Those involving deep sites are relatively rare. To the best of our knowledge, only one case of primary intraosseous hybrid peripheral nerve sheath tumours has been documented. In this article, we report another case of hybrid peripheral nerve sheath tumours occurring in bone with different clinical, radiological and pathological features from those in the previously reported cases. Case presentation A 28-year-old female presented with a painful nodule in the right tibia. Radiological examination revealed an oval eccentric osteolytic lesion in the proximal tibia. Histologically, the circumscribed but unencapsulated lesion demonstrated biphasic cellular differentiation. Bland, small epithelioid cells arranged in clusters in the myxoid or collagenous stroma and inconspicuous spindle cells scattered in the hypercellular areas were suggested to originate from Schwann cells according to the detection of S100. Both the elongated spindle cells with thin, wavy nuclei and the spindle cells in fascicular or storiform pattern in hypercellular areas showed a positive immunoreaction for epithelial membrane antigen, indicating perineurial differentiation. Based on histological and immunochemical examinations, the patient was diagnosed with hybrid epithelioid schwannoma/perineurioma. The lesion was resected and has not recurred for 8 months since resection. Conclusion The present case is the second primary intraosseous hybrid peripheral nerve sheath tumour to be reported. This is also the first reported intraosseous tumour composed of epithelioid schwannoma and perineurioma with hypercellularity, indicating diverse involvement sites and a wide range of histological features among hybrid peripheral nerve sheath tumours. Awareness of such diversity is critical for accurate diagnoses. The morphological overlap with other spindle and epithelioid cell neoplasms, especially pure peripheral nerve sheath tumours, requires that immunochemical and molecular examinations be used as objective tools to provide the necessary information for a differential diagnosis.http://link.springer.com/article/10.1186/s13000-019-0829-xHybrid peripheral nerve sheath tumoursEpithelioid schwannomaPerineuriomaIntraosseous neoplasmHypercellularity |
| spellingShingle | Yuejiao Lang Dawei Liu Pei Xiang Jilin Wang Yang Li Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity Diagnostic Pathology Hybrid peripheral nerve sheath tumours Epithelioid schwannoma Perineurioma Intraosseous neoplasm Hypercellularity |
| title | Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity |
| title_full | Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity |
| title_fullStr | Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity |
| title_full_unstemmed | Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity |
| title_short | Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity |
| title_sort | primary intraosseous hybrid epithelioid schwannoma perineurioma in the proximal tibia a case report of benign hybrid neoplasm with local hypercellularity |
| topic | Hybrid peripheral nerve sheath tumours Epithelioid schwannoma Perineurioma Intraosseous neoplasm Hypercellularity |
| url | http://link.springer.com/article/10.1186/s13000-019-0829-x |
| work_keys_str_mv | AT yuejiaolang primaryintraosseoushybridepithelioidschwannomaperineuriomaintheproximaltibiaacasereportofbenignhybridneoplasmwithlocalhypercellularity AT daweiliu primaryintraosseoushybridepithelioidschwannomaperineuriomaintheproximaltibiaacasereportofbenignhybridneoplasmwithlocalhypercellularity AT peixiang primaryintraosseoushybridepithelioidschwannomaperineuriomaintheproximaltibiaacasereportofbenignhybridneoplasmwithlocalhypercellularity AT jilinwang primaryintraosseoushybridepithelioidschwannomaperineuriomaintheproximaltibiaacasereportofbenignhybridneoplasmwithlocalhypercellularity AT yangli primaryintraosseoushybridepithelioidschwannomaperineuriomaintheproximaltibiaacasereportofbenignhybridneoplasmwithlocalhypercellularity |