scholarly journals Update on Superficial Spindle Cell Mesenchymal Tumors in Children

2021 ◽  
Vol 8 (3) ◽  
pp. 285-300
Author(s):  
Philippe Drabent ◽  
Sylvie Fraitag
Keyword(s):  
Spindle Cell ◽  
Dermatofibrosarcoma Protuberans ◽  
Diagnostic Value ◽  
Mesenchymal Tumors ◽  
Infantile Fibrosarcoma ◽  
Genetic Features ◽  
Fibrous Hamartoma Of Infancy ◽  
Neural Tumor ◽  
Prognostic Implications ◽  
Plexiform Fibrohistiocytic Tumor

The diagnosis of cutaneous and subcutaneous spindle cell neoplasms in children is often challenging and has potential therapeutic and prognostic implications. Although correctly diagnosing dermatofibrosarcoma protuberans and infantile fibrosarcoma is paramount, pathologists should not ignore a number of diagnostic pitfalls linked to mostly rare tumors with completely different clinical outcomes. In the last decade, a spectrum of novel entities has been described; information from molecular biology has helped to shape this new landscape for spindle cell tumors. Here, we review the most noteworthy neoplasms in this spectrum, with a focus on their histological similarities: fibroblastic connective tissue nevus, medallion-like dermal dendrocyte hamartoma, or plaque-like CD34-positive dermal fibroma, which share features with fibrous hamartoma of infancy; lipofibromatosis and lipofibromatosis-like neural tumor; and plexiform myofibroblastoma, a recently described neoplasm that should be distinguished from plexiform fibrohistiocytic tumor. These tumors also have genetic similarities, particularly gene rearrangements involving NTRK3 or NTRK1. These genetic features are not only essential for the differential diagnosis of infantile fibrosarcoma but are also of diagnostic value for lipofibromatosis-like neural tumors. The more recently described RET, RAF1, and BRAF gene fusions are also discussed.

scholarly journals Fibroblastic and myofibroblastic tumors of children: new genetic entities and new ancillary testing

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 1963 ◽  
Author(s):  
David M Parham
Keyword(s):  
Differential Diagnosis ◽  
Spindle Cell ◽  
Dermatofibrosarcoma Protuberans ◽  
Infantile Myofibromatosis ◽  
Unique Biology ◽  
Fibrous Hamartoma Of Infancy ◽  
Age Range ◽  
Variable Group ◽  
Calcifying Aponeurotic Fibroma ◽  
Infants And Young Children

Fibroblastic and myofibroblastic tumors comprise a morphologically diverse and biologically variable group of neoplasms that affect a wide age range. Specific entities tend to occur most frequently in infants and young children. Recent years have witnessed a proliferation of information concerning the unique biology of these tumors. In this report, I will review recent findings that serve to further characterize this group of neoplasms. Included will be newer information on fibrous hamartoma of infancy, infantile myofibromatosis, lipofibromatosis, and infantile fibrosarcoma and tumors resembling it, including primitive myxoid mesenchymal tumor of infancy and new genetic entities. I will also discuss the differential diagnosis, which includes spindle cell rhabdomyosarcoma, dermatofibrosarcoma protuberans, and calcifying aponeurotic fibroma.

Diagnostic Value of Electron Microscopy in Soft Tissue Tumors

1970 ◽  
Vol 28 ◽  
pp. 220-221
Author(s):  
Gerald Fine ◽  
Azorides R. Morales
Keyword(s):  
Cardiac Myxoma ◽  
Osteogenic Sarcoma ◽  
Diagnostic Value ◽  
Soft Tissue Tumors ◽  
Amelanotic Melanoma ◽  
Growth Patterns ◽  
Light Microscopic Level ◽  
Mesenchymal Tumors ◽  
Good Agreement

For years the separation of carcinoma and sarcoma and the subclassification of sarcomas has been based on the appearance of the tumor cells and their microscopic growth pattern and information derived from certain histochemical and special stains. Although this method of study has produced good agreement among pathologists in the separation of carcinoma from sarcoma, it has given less uniform results in the subclassification of sarcomas. There remain examples of neoplasms of different histogenesis, the classification of which is questionable because of similar cytologic and growth patterns at the light microscopic level; i.e. amelanotic melanoma versus carcinoma and occasionally sarcoma, sarcomas with an epithelial pattern of growth simulating carcinoma, histologically similar mesenchymal tumors of different histogenesis (histiocytoma versus rhabdomyosarcoma, lytic osteogenic sarcoma versus rhabdomyosarcoma), and myxomatous mesenchymal tumors of diverse histogenesis (myxoid rhabdo and liposarcomas, cardiac myxoma, myxoid neurofibroma, etc.)

scholarly journals Dermatofibrosarcoma protuberans: a tumor in the wide spectrum of the bland-looking spindle cell lesions of the breast

Pathologica ◽  
2019 ◽  
Vol 111 (3) ◽  
pp. 87-91
Author(s):  
G. M. Vecchio ◽  
G. Broggi ◽  
A. Mulè ◽  
E. Piombino ◽  
G. Magro
Keyword(s):  
Spindle Cell ◽  
Wide Spectrum ◽  
Dermatofibrosarcoma Protuberans

Immunohistochemical Expression of Matrix Metalloproteinases 1, 2, 9, and 14 in Dermatofibrosarcoma Protuberans and Common Fibrous Histiocytoma (Dermatofibroma)

2004 ◽  
Vol 128 (10) ◽  
pp. 1136-1141 ◽  
Author(s):  
David M. Weinrach ◽  
Kim L. Wang ◽  
Elizabeth L. Wiley ◽  
William B. Laskin
Keyword(s):  
Matrix Metalloproteinases ◽  
Tumor Cells ◽  
Fibrous Histiocytoma ◽  
Dermatofibrosarcoma Protuberans ◽  
Growth Patterns ◽  
Dermal Fibroblasts ◽  
Major Constituent ◽  
Mesenchymal Tumors ◽  
Formalin Fixed Paraffin Embedded ◽  
Immunohistochemical Studies

Abstract Context.—Common fibrous histiocytoma (cFH) or dermatofibroma and dermatofibrosarcoma protuberans (DFSP) are 2 spindle cell mesenchymal tumors that are distinguished in part by their microscopic growth patterns and clinically by the greater propensity for DFSP to recur. Matrix metalloproteinases (MMPs) potentially play a role in modulating the growth patterns of cFH and DFSP by remodeling the extracellular matrix. Objective.—To evaluate the immunohistochemical (IHC) expression of MMP-1, MMP-2, MMP-9, and MMP-14 in DFSP and cFH, because (1) MMP-1, MMP-2, MMP-9, and MMP-14 are synthesized by dermal fibroblasts, the major constituent of DFSP and cFH; and (2) platelet-derived growth factor B, which is overexpressed in most examples of DFSP because of t(17;22), activates ets-1, a transcription factor that regulates molecules associated with tumor invasion and metastasis, including MMP-1, MMP-3, and MMP-9. Design.—Immunohistochemical studies were performed on archived, formalin-fixed, paraffin-embedded tissue of DFSP (n = 48) and cFH (n = 47). Results.—Significant IHC expression (>10% of tumor cells) in cFH included MMP-14 (27 [59%] of 46 tumors positive), MMP-2 (21 [47%] of 45 tumors positive), MMP-9 (9 [20%] of 45 tumors positive), and MMP-1 (6 [13%] of 46 tumors positive). No DFSPs showed significant IHC expression of any of the MMPs evaluated. However, anti– MMP-2 highlighted a rich microvascular element within deep tumor tissue present in 81% of DFSPs with a prominent subcutaneous component. Conclusion.—Our IHC results indicate that MMP-1 and MMP-9 are not up-regulated in DFSP. Convincing expression of MMP-14 in cFH suggests that this MMP may affect the growth pattern of the lesion, perhaps by activating MMP-2 expression in tumor cells. In DFSP, MMP-2 may play a role in tumor angiogenesis.

scholarly journals Angiomyolipoma of colon: unusual presentation

2019 ◽  
Vol 6 (6) ◽  
pp. 2204
Author(s):  
Vidhyachandra Gandhi ◽  
Swapnil Karnik ◽  
Nitin Pai ◽  
Sujai Hegde
Keyword(s):  
Spindle Cell ◽  
Histopathological Examination ◽  
Cell Type ◽  
Mesenchymal Tumors ◽  
Left Hemicolectomy ◽  
Mature Adipose Tissue ◽  
Polypoidal Lesion ◽  
Descending Colon ◽  
One Year ◽  
Kidney Angiomyolipoma

Angiomyolipomas are benign mesenchymal tumors mostly arising from the kidney. Angiomyolipoma of the colon is extremely rare. Here we report the findings of a 72 years gentleman who presented with recurrent episodes of abdominal pain and fullness of one year duration. Colonoscopy was suggestive of polypoidal lesion in the descending colon. CECT abdomen revealed a colocolic intussusception in the descending colon with lipoma as a leading point. He underwent a standard left hemicolectomy. Histopathological examination showed that the tumor of 5.7 cm in diameter included smooth muscle (spindle cell type), mature adipose tissue, and vessels, and therefore a diagnosis of angiomyolipoma was made. We believe this is the second report of colonic angiomyolipoma presenting with colocolic intussusception. 

scholarly journals Diagnostic Challenges of Dermatofibrosarcoma Protuberans (DFSP), a Rare Spindle Cell Tumor of Breast

Cureus ◽  
2021 ◽  
Author(s):  
Sanobar Yasmeen Mohammed ◽  
Qandeel Sadiq ◽  
David Mcgregor ◽  
Farhan Khan
Keyword(s):  
Spindle Cell ◽  
Dermatofibrosarcoma Protuberans ◽  
Cell Tumor ◽  
Spindle Cell Tumor

scholarly journals Gastrointestinal stromal tumors (gists): Definition, clinical, histological, immunohistochemical and molecular genetic features and predictors of malignant potential and differential diagnosis

2002 ◽  
Vol 10 (4) ◽  
pp. 267-271 ◽  
Author(s):  
Vesna Zivkovic ◽  
Vuka Katic ◽  
Aleksandar Nagorni ◽  
Ljubinka Velickovic ◽  
Maja Milentijevic ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Molecular Genetic ◽  
Malignant Potential ◽  
Gi Tract ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Cellular Origin ◽  
Genetic Features ◽  
Exon 11 ◽  
Malignant Gists

Gastrointestinal stromal tumors (GISTs) represent a distinct and the most important subset of mesenchymal tumors of the gastrointestinal (GI) tract GISTs occur throughout the GI tract but are usually located in the stomach and small intestine. The cellular origin, differentiation, nomenclature and prognosis of GISTs are controversial. Because GISTs, like the interstitial cells of Cajal, the GI pacemaker cells, express CD117 (c-kit protein), the origin of GISTs from the Cajal cells has recently been suggested. GISTs are also known for their wide variability in clinical behavior and for the difficulty to determine their malignant condition The most reproducible predictors of malignancy are mitotic count >1-5 per10 high-powered fields (HPF), size >5 cm, tumor necrosis, infiltration and metastasis to other sites. However, some tumors with mitotic activity <1/10 HPF may metastasize indicating some uncertainty in malignant potential of GISTs, especially those larger than 5 cm. Recently, mutations in c-kit gene (exon 11) preferentially occur in malignant GISTs and may be a clinically useful adjunct marker in evaluation of GISTs. In conclusion, the strong CD117 expression mostly defines primary GI mesenchymal tumors as GIST. Specific identification of GIST may become clinically important if therapies targeting the c-kit tyrosine kinase activation become available.

scholarly journals Immunohistochemical features of gastrointestinal stromal tumors and their role for differential diagnosis and prognosis

2021 ◽  
pp. 10-16
Author(s):  
Yana Miroshnichenko
Keyword(s):  
Differential Diagnosis ◽  
Tumor Cells ◽  
Gastrointestinal Stromal Tumors ◽  
Spindle Cell ◽  
Prognostic Markers ◽  
Mitotic Rate ◽  
Mesenchymal Tumors ◽  
Ki 67 ◽  
Stromal Tumors ◽  
Strong Expression

The aim. To clarify all most important immunohistochemical features of gastrointestinal stromal tumors with different histological patterns and analyze the role of expression of Ki-67, MMP-9, VEGF and p16ink4A as a predictive markers of tumor progression. Materials and methods. The study is based on analysis of 100 primary GISTs for description of their morphological features and 36 GISTs taken from this 100 for study of prognostic markers. Results. All spindle cell GISTs have shown diffuse expression of CD117 in tumor cells. The levels of CD117 expression varied from strong expression (3+) until mild expression (1+). Strong expression were seen in 75,8 % of spindle cell GISTs. Epithelioid GISTs demonstrated heterognous moderate or mild expression of CD117. All primary epithelioid GISTs from patients that had relapse of tumor in period from 1 till 3 years demonstrated focal mild expression of CD 117 in tumor cells. Expression of DOG-1 were seen in all 100 cases of GISTs, that were included in our study. The strong expression of DOG-1 (3+) were seen in all 45 GISTs that had low mitotic rate (≤5 mitoses per 50HPF) and not associated with their histological pattern. GISTs with high mitotic rate demonstrated heterogeneous expression of DOG-1 in tumors: moderate expression (2+) with patchy areas of strong expression (3+). Expression of CD56 was not found in spindle cell GISTs, but single tumor cells of epithelioid GISTs that had high mitotic rate demonstrated expression of this marker. The average expression of p16ink4A were higher in tumors that gave relapses compared with tumors without relapses (50,3 % versus 5,7 % respectively, U-test=16.5; p≤0,01).The average expression of MMP-9 also were significantly higher in GISTs that gave relapses: 63,2 % compared with 13,4 % in GISTs without relapse (U-test=16; p≤0 ,01).The strong VEGF expression was found in 66,7 % of GISTs that had relapses and only in 8,3 % of GISTs without relapses. 50 % of GISTs without relapses was negative for VEGF. Finally, the average expression of Ki-67 were 13,4 % in GISTs with relapses and 8,7 % in GISTs without them (U-test=16; p≤0,01). Conclusion. We highly recommend using DOG-1 for epithelioid GISTs. Additionally in epithelioid GISTs can be used CD56 that can give focal positive reaction in some tumour cells. The following minimal panel of markers for differential diagnosis of spindled GISTs from other mesenchymal tumors of gastrointestinal tract is proposed: CD117, DOG-1 and SMA, where the first too markers will demonstrated the moderate or strong diffuse expression and SMA can be occasionally positive in some tumor cells. p16ink4A, ki-67, VEGF and MMP-9 can be used as additional prognostic markers in GISTs.

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