EWSR1-SMAD3-Positive Fibroblastic Tumor

2020 ◽  
pp. 106689692093812
Author(s):  
Oliver Foot ◽  
Magnus Hallin ◽  
Robin L. Jones ◽  
Vaiyapuri P. Sumathi ◽  
Khin Thway
Keyword(s):  
Spindle Cell ◽  
Soft Tissue Tumors ◽  
Spindle Cell Sarcoma ◽  
Correct Identification ◽  
Nerve Sheath Tumor ◽  
Not Otherwise Specified ◽  
Nerve Sheath ◽  
Spindle Cells ◽  
Subcutaneous Tissues ◽  
Malignant Soft Tissue

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.

A Rare Cause of Primary Hepatic Neoplasm: Malignant Peripheral Nerve Sheath Tumor in the Age of Modern Liver Surgery

2008 ◽  
Vol 74 (1) ◽  
pp. 47-50 ◽  
Author(s):  
Douglas M. Iddings ◽  
Byron E. Wright ◽  
Anton Bilchik
Keyword(s):  
Peripheral Nerve ◽  
Biopsy Specimen ◽  
Spindle Cell ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Spindle Cells ◽  
Clinical Diagnoses ◽  
Impact On Treatment ◽  
Hepatic Neoplasm

Primary malignant peripheral nerve sheath tumor (MPNST) of the liver is rare. Histologic identification of spindle cells from a biopsy specimen and the potential clinical diagnoses are discussed. Potential metastatic and primary spindle cell lesions, as well as their impact on treatment decisions are considered. This was successfully treated with ablation assisted surgical resection and minimal blood loss.

A Case Report Of A Spindle Cell Sarcoma With Ntrk-3 Rearrangement And A Novel Gene Fusion Partner (Ntrk3-Eif2s2)

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S69-S70
Author(s):  
A Shalaby ◽  
A Alhussain ◽  
Y Al Hmada ◽  
A Bernieh
Keyword(s):  
Gene Fusion ◽  
Spindle Cell ◽  
Fusion Gene ◽  
Spindle Cell Sarcoma ◽  
Fusion Partner ◽  
Nerve Sheath Tumor ◽  
Sequencing Analysis ◽  
Cell Sarcoma ◽  
Infantile Fibrosarcoma ◽  
Spindle Cells

Abstract Casestudy A group of spindle cell tumors with characteristic morphologic features, co-expression of CD34 and S100 and recurrent gene rearrangements in RAF-1, BRAF, and NTRK has recently been described. These tumors were found to be previously labeled as malignant peripheral nerve sheath tumor, infantile fibrosarcoma or unclassified spindle cell sarcoma. We describe a case of a 25-year-old female who presented with a right thigh mass. She underwent an ultrasound-guided biopsy showing a spindle cell tumor with co-expression of CD34 and S100 and subsequently underwent resection of the mass. Gross examination showed a 7.5 cm multi-lobulated, tan-pink, hemorrhagic and fleshy mass. Histologically, the tumor was relatively well-demarcated and consisted of spindle cells with moderate to high cellularity in a patternless architecture. The spindle cells showed moderate to marked pleomorphism, pale amphophilic cytoplasm, ovoid-to-elongated nuclei with vesicular chromatin, and occasional prominent nucleoli. Areas of prominent perivascular and stromal hyalinization were seen. Mitotic activity was brisk with up to 33 mitoses per 10 high power fields. Necrosis representing approximately 5% of the mass was identified. On immunohistochemistry, the tumor cells showed strong and diffuse positivity for CD34 and S100 and were negative for SOX10, broad-spectrum cytokeratin, EMA, SMA, Desmin, STAT6, MUC4, TLE1, and H3K27me3 (retained nuclear expression). EIF2S2-NTRK3 fusion gene was detected using next generation sequencing analysis. Conclusion A few cases of NTRK3 spindle cell sarcomas, other than classic infantile fibrosarcoma, have been previously reported in the literature with fusion genes involving ETV6, EML4, and STRN, among others. A gene fusion involving NTRK3 and EIF2S2 has not been previously reported. NTRK3-fused sarcomas typically show high-grade morphology and aggressive clinical behavior. Identification of NTRK-fused sarcomas is clinically important, as these advanced tumors are potentially amenable to NTRK inhibition. In our case, patient received adjuvant post-operative radiation therapy and returned with lung metastasis 5 months after surgery.

scholarly journals Bovine Papillomavirus DNA and S100 Profiles in Sarcoids and Other Cutaneous Spindle Cell Tumors in Horses

2016 ◽  
Vol 54 (1) ◽  
pp. 44-52 ◽  
Author(s):  
E. D. Epperson ◽  
W. L. Castleman
Keyword(s):  
Soft Tissue ◽  
Spindle Cell ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors ◽  
Bovine Papillomavirus ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Spindle Cell Tumors ◽  
Tumor Types

Histopathologic differentiation between deep dermal or subcuticular equine sarcoids (ie, nodular sarcoids) and other spindle cell tumors in the dermis and subcutis such as peripheral nerve sheath tumors (PNSTs) can be challenging based on morphologic criteria alone. It has been proposed that polymerase chain reaction (PCR) for bovine papillomavirus (BPV) DNA and S100 immunohistochemistry be used as diagnostic tests to separate equine sarcoids from PNSTs. We reviewed 197 skin-associated spindle cell tumors (ie, soft tissue sarcomas), including PNSTs and sarcoids, received at the University of Florida between 1995 and 2013 and performed BPV PCR and S100 immunohistochemistry on archived paraffin-embedded tissues. We found that BPV DNA was demonstrable in 70% of the sarcoids, 59% of the PNSTs, 37% of the fibrosarcomas, and 22% of other tumors (myxosarcomas, fibromas, and other sarcomas) diagnosed on histomorphologic characteristics. Positive S100 staining was only seen in 12 tumors in the study (5 fibrosarcomas, 3 sarcoids, 2 PNSTs, and 2 other sarcomas). The results demonstrate that BPV is associated with many skin-associated spindle cell soft tissue tumors in horses in addition to sarcoids. S100 was rarely expressed in equine soft tissue sarcomas in the skin but was expressed in many tumor types, including PNSTs and sarcoids. Because 41% of the PNSTs classified by histomorphology in this study were BPV negative and 94% were S100 negative, it is reasonable to classify these as soft tissue sarcomas with nerve sheath tumor histomorphology rather than as either PNSTs or sarcoids.

scholarly journals Metastatic small cell carcinoma of the lung with prominent spindle cell morphology and hemangiopericytoma-like vascular pattern: A sarcoma mimicker

2020 ◽  
Vol 8 ◽  
pp. 2050313X2098117
Author(s):  
Esra Nsour ◽  
Ali Al Khader ◽  
Bushra Al-Tarawneh
Keyword(s):  
Cell Carcinoma ◽  
Small Cell Carcinoma ◽  
Spindle Cell ◽  
Small Cell ◽  
Nerve Sheath Tumor ◽  
Vascular Pattern ◽  
Nerve Sheath ◽  
History Of ◽  
Wall Mass ◽  
Aggressive Clinical Behavior

Small cell carcinoma is a malignant neuroendocrine tumor with aggressive clinical behavior. Histologically, the tumor is characterized by the proliferation of small, round, blue cells. Here, we present the case of a 50-year-old man with a 1-month history of enlarging chest wall mass. Microscopic examination of the lesion revealed a highly cellular neoplasm composed of closely packed, atypical spindle cells with scant cytoplasm, inconspicuous nucleoli, and brisk mitotic activity. The hemangiopericytoma-like vascular pattern was prominent. Areas showing a fibrosarcoma-like fascicular pattern were also observed. The tumor was immunohistochemically positive for TTF1, synaptophysin, and chromogranin, confirming small cell carcinoma. Further investigations revealed a lung origin and widespread metastases. The tumor in this case closely mimicked synovial sarcoma or malignant peripheral nerve sheath tumor. Small cell carcinoma demonstrates a hemangiopericytoma-like pattern that can mimic sarcoma histologically. This is a serious pitfall that can significantly affect the speed of diagnosis and management.

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
Keyword(s):  
Immunohistochemical Study ◽  
Correct Diagnosis ◽  
S100 Protein ◽  
Soft Tissue Tumors ◽  
Biological Behavior ◽  
Ultrastructural Examination ◽  
Spindle Cells ◽  
Nasal Chondromesenchymal Hamartoma ◽  
Myofibroblastic Differentiation ◽  
Malignant Soft Tissue

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.

scholarly journals Malignant peripheral nerve sheath tumor of the distal phalanx of the fifth toe: a case report

2014 ◽  
Vol 3 (1) ◽  
pp. 204798161351603 ◽  
Author(s):  
Yuki Iwama ◽  
Makoto Kunisada ◽  
Hajimu Goto ◽  
Yoshiharu Ohno ◽  
Junji Yamashita ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Distal Phalanx ◽  
Nerve Sheath Tumor ◽  
Resonance Imaging ◽  
Peripheral Nerve Sheath Tumor ◽  
Histological Features ◽  
Nerve Sheath ◽  
Subcutaneous Tissues ◽  
Fifth Toe ◽  
The Right

Malignant peripheral nerve sheath tumor (MPNST) involving bone is rare. We report a case of MPNST of the fifth toe. The lesion was located in the distal phalanx of the right fifth toe and extended into surrounding subcutaneous tissues. Findings on magnetic resonance imaging and histological features of the case are described and the literature is briefly reviewed.

Recurrence of acoustic neurilemoma as a malignant spindle-cell neoplasm

1990 ◽  
Vol 73 (6) ◽  
pp. 946-950 ◽  
Author(s):  
Catriona A. McLean ◽  
John D. Laidlaw ◽  
David S. B. Brownbill ◽  
Michael F. Gonzales
Keyword(s):  
Cerebellopontine Angle ◽  
Spindle Cell ◽  
Positive Staining ◽  
Nerve Sheath Tumor ◽  
Content Type ◽  
Spindle Cell Neoplasm ◽  
Nerve Sheath ◽  
Cell Neoplasm ◽  
Acoustic Nerve ◽  
S 100

✓ A 75-year-old man presented with a right cerebellopontine angle tumor 11 months after complete macroscopic resection of a right acoustic neurilemoma. Histological examination of the recurrent tumor showed a malignant spindle-cell neoplasm with positive staining for S-100 protein. The patient had no stigmata of von Recklinghausen's disease. It is proposed that this recurrence represents progression from a benign to a malignant acoustic nerve-sheath tumor, an event that is extremely rare outside the clinicopathological context of neurofibromatosis.

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