Pediatric Undifferentiated Sarcoma of the Soft Tissues: A Clinicopathologic Study

Primitive round- or spindle-cell EWSR1-negative undifferentiated sarcomas harboring CIC-DUX4 gene fusion are the most common form of Ewing-like sarcomas. These tumors primarily occur in peripheral soft tissues, but examples have been described within viscera and the brain. As far as we are aware, CIC-DUX4 positive primary epidural spinal sarcoma has not been reported. Herein, we describe a T5–T6 epidural tumor in a 15-year-old girl in which many neoplastic cells had moderate and focally abundant cytoplasm, including plasmacytoid or rhabdoid cells, rather than the more common Ewing-like morphology described in the majority of such tumors. The diagnosis was confirmed by fluorescent in situ hybridization after the tumor was found to be WT-1 positive, and comprehensive genomic profiling demonstrated breakpoints in exon 20 and exon 1 of the CIC and DUX4 genes, respectively. After treatment with local radiation and systemic chemotherapy, resected recurrent tumor demonstrated more pleomorphic neoplastic cells as well as intracytoplasmic eosinophilic globules and nuclear pseudoinclusions which may reflect therapy-related changes. Unfortunately, there was further progression of tumor including the development of intracranial lesions, and the patient succumbed to her tumor 22 months after the original resection.

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Rhabdoid tumors of soft tissues: A clinicopathologic study of 26 cases enrolled on the intergroup rhabdomyosarcoma study

Human Pathology ◽

10.1016/0046-8177(91)90289-2 ◽

1991 ◽

Vol 22 (7) ◽

pp. 674-684 ◽

Cited By ~ 127

Author(s):

Roman Kodet ◽

William A. Newton ◽

Nancy Sachs ◽

Ala B. Hamoud ◽

R. Beverly Raney ◽

...

Keyword(s):

Soft Tissues ◽

Clinicopathologic Study ◽

Rhabdoid Tumors

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Primary Renal BCOR-CCNB3 Fusion Sarcoma: A Case Report and Review of the Literature

Urologia Internationalis ◽

10.1159/000518563 ◽

2021 ◽

pp. 1-4

Author(s):

Ming Zhao ◽

Qi Zhang ◽

Xianglei He ◽

Dahong Zhang

Keyword(s):

Case Report ◽

Soft Tissues ◽

Clinical Course ◽

Accurate Diagnosis ◽

Review Of The Literature ◽

Undifferentiated Sarcoma

BCOR-CCNB3 fusion sarcoma is a recently described undifferentiated sarcoma with a novel recurrent inversion of 2 nearby genes BCOR and CCNB3. It typically affects bone and soft tissues of the pelvis, extremity, and paraspinal region and pursues variable clinical course. Primary renal BCOR-CCNB3 fusion sarcoma is very rare, and only a small number of cases have been documented. Accurate diagnosis is often challenging, and there is not any agreement for the treatment of this entity due to its rarity. We report findings of primary renal BCOR-CCNB3 fusion sarcoma in a 16-year-old boy with a brief review of the literature.

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Undifferentiated sarcoma of the soft tissues with cystic degeneration: Case report and review of literature

Journal of Cancer Research and Therapeutics ◽

10.4103/jcrt.jcrt_818_18 ◽

2019 ◽

Vol 15 (4) ◽

pp. 947 ◽

Cited By ~ 1

Author(s):

Lei Guo ◽

Changhua Wu ◽

Liang Wang ◽

Lihong Zhang ◽

Jing Li

Keyword(s):

Case Report ◽

Soft Tissues ◽

Cystic Degeneration ◽

Review Of Literature ◽

Undifferentiated Sarcoma

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Deep Granuloma Annulare (Pseudorheumatoid Nodule) in Children: Clinicopathologic Study of 35 Cases

Pediatric and Developmental Pathology ◽

10.1007/s100249900043 ◽

1998 ◽

Vol 1 (4) ◽

pp. 300-308 ◽

Cited By ~ 39

Author(s):

M.B. McDermott ◽

A.C. Lind ◽

E.F. Marley ◽

L.P. Dehner

Keyword(s):

Soft Tissues ◽

Mononuclear Cells ◽

Epithelioid Sarcoma ◽

Granuloma Annulare ◽

Clinicopathologic Study ◽

Eosinophilic Cytoplasm ◽

Anterior Tibial ◽

Subcutaneous Mass ◽

Characteristic Features

Deep granuloma annulare (DGA) is one of several lesions of skin and superficial soft tissues whose histologic character is a palisading granuloma with a small central focus of necrosis or necrobiosis. Unlike the other palisading necrobiotic lesions, DGA has a predilection for children in the first 5 to 6 years of life. A painless subcutaneous nodule(s) in the lower anterior tibial region or foot and the scalp, typically in the occiput, was the most common presenting feature in this study of 35 cases. Additional or recurrent lesions were reported in approximately 70% of cases with clinical follow-up. All lesions showed the presence of necrobiosis; however, one of the characteristic features was the multinodular character of the predominantly mononuclear cellular aggregates. The presence of vascular spaces at the periphery of the nodular profiles served as a clue to the diagnosis of DGA. The palisading arrangement of the mononuclear cells was evident only in those foci with central necrobiosis. A histiocytic disorder or fibrohistiocytic process was a common consideration in the differential diagnosis, especially in those cases with less apparent foci of necrosis. Palisading histiocytes with prominent eosinophilic cytoplasm and some nuclear atypism were problematic with regard to possible epithelioid sarcoma. Our study failed to identify any underlying or predisposing factors in the development of DGA. Despite the fact that DGA is a well-documented lesion in children, it occurs sufficiently infrequently that it is often not considered clinically when it presents as a subcutaneous mass or masses in a child. Its recognition by the pathologist is especially important as the occurrence of additional lesions in a high proportion of children can be anticipated without undue concern.

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Undifferentiated sarcoma: does it exist? A clinicopathologic study of 7 pediatric cases and review of literature

Human Pathology ◽

10.1016/j.humpath.2009.04.013 ◽

2009 ◽

Vol 40 (11) ◽

pp. 1600-1610 ◽

Cited By ~ 36

Author(s):

Rita Alaggio ◽

Gianni Bisogno ◽

Antonio Rosato ◽

Vito Ninfo ◽

Cheryl M. Coffin

Keyword(s):

Review Of Literature ◽

Undifferentiated Sarcoma ◽

Clinicopathologic Study

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Clinicopathologic Study of Ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV

Pediatric and Developmental Pathology ◽

10.1007/s100249910039 ◽

2000 ◽

Vol 3 (3) ◽

pp. 290-300 ◽

Cited By ~ 31

Author(s):

Daniel R. Boué ◽

David M. Parham ◽

Bruce Webber ◽

William M. Crist ◽

Stephen J. Qualman ◽

...

Keyword(s):

Ganglion Cells ◽

Prognostic Significance ◽

Neuron Specific Enolase ◽

Study Groups ◽

Malignant Neoplasms ◽

Membrane Expression ◽

Undifferentiated Sarcoma ◽

Clinicopathologic Study ◽

Microscopic Features ◽

Primary Neoplasm

Ectomesenchymomas (EM) are rare malignant neoplasms usually consisting of rhabdomyosarcoma (RMS) with a neural component. Only 21 cases have been previously reported. Here we extend the clinicopathologic spectrum of EM by describing our findings in 15 cases. Only 5 patients were infants; 10 were ≤3 years old and 5 were ≥6 years old. No male predilection was observed; 7 were female. The originating institutional diagnoses were; RMS (12), undifferentiated sarcoma (1), or EM (2), suggesting underdiagnosis of this entity. The primary tumor sites included external genital (5), pelvis/abdomen (6), head and neck (3), and extremity (1). The size of the primary neoplasm was usually ≥5 cm at diagnosis but dissemination only occurred in a minority. Local infiltration was not uncommon. These neoplasms were typically multilobate, thinly encapsulated, hemorrhagic, and necrotic. Light microscopic features were highly variable, but embryonal RMS with scattered or clustered ganglion cells, often in lacunae, was characteristic. In some cases, primitive neuroblastic or neuroectodermal areas were found and/or a component of alveolar RMS was seen. Focal anaplasia was occasionally observed. Mitotic activity appears higher than previously appreciated and some necrosis was invariably present. Electron microscopy was performed in 11 cases, which confirmed skeletal muscle ± neural differentiation. Cytogenetic studies performed in five cases revealed no specific abnormality. Monoclonal neuron-specific enolase was the best marker of ganglion cells and primitive neural elements. MIC-2 (CD99) membrane expression was not definitively present in any of the six cases examined. A number of the above parameters appear to be of some prognostic significance, but overall, these neoplasms appear to have a similar outcome as would be predicted for their RMS element alone (exclusive of any neural component), with respect to the RMS subtype, age of the patient, and anatomic location of the neoplasm.

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Canine Extraskeletal Osteosarcoma and Chondrosarcoma: a Clinicopathologic Study of 14 Cases

Veterinary Pathology ◽

10.1177/030098589002700107 ◽

1990 ◽

Vol 27 (1) ◽

pp. 46-55 ◽

Cited By ~ 85

Author(s):

A. K. Patnaik

Keyword(s):

Soft Tissues ◽

Single Case ◽

Medical Center ◽

Distant Metastases ◽

Human Beings ◽

Extraskeletal Osteosarcoma ◽

Mitral Valves ◽

Clinicopathologic Study ◽

The Mean ◽

Chondroblastic Osteosarcoma

Canine extraskeletal osteosarcomas are extremely rare tumors. Over a period of 25 years at the Animal Medical Center, approximately 1,000 cases of skeletal osteosarcomas have been diagnosed. During the same period 11 cases of extraskeletal osteosarcomas and three extraskeletal chondrosarcomas were diagnosed. Tumors of the mammary gland were excluded. Extraskeletal osteosarcomas were found in the adrenal gland, eye, gastric ligament, ileum, kidney, liver, spleen, testicle, and vagina. The chondrosarcomas were found in the mitral valves, lungs, and omentum. The mean age of the dogs with extraskeletal osteosarcoma was 11 years, and the mean age of the dogs with extraskeletal chondrosarcoma was 14 years. The sizes of the tumors ranged from 3 cubic centimeters to 8,315 cubic centimeters. Osteoblastic osteosarcomas were the most common histologic type (7/11, 63.6%); there was a single case of each of the following: fibroblastic, fibrous histiocytic, chondroblastic, and mixed osteo-chondroblastic osteosarcoma. Two of the dogs with chondrosarcomas had mesenchymal chondrosarcomas involving the lungs and omentum. The remaining dog had a regular chondrosarcoma involving the mitral valve. Distant metastases were present in seven of 11 dogs with extraskeletal osteosarcoma and in none of the dogs with chondrosarcoma. In contrast to human beings, in which most extraskeletal osteosarcomas occur in the soft tissues and the extremities, most canine extraskeletal osteosarcomas develop in the visceral organs.

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