Synchronous Presentation of an Askin Tumor and a Plasmacytoma in an Adult Patient

Askin tumor, or malignant small round cell tumor of the thoracopulmonary region, is an extremely infrequent entity occurring primarily in children and adolescents. Its histopathologic and cytogenetic features suggest that it belongs to the family of Ewing's sarcoma and primitive neuroectodermal tumors. We report the case of a 43-year-old woman affected by an Askin tumor with bone metastases at diagnosis, presenting synchronously with a plasmacytoma. This is the first reported case of the simultaneous occurrence of an Askin tumor and a malignant hemopathy. The progression of the former and the remission of the plasmacytoma during chemotherapy were remarkable, since Askin tumor treatment shares drugs used for the treatment of plasma cell tumors. Given the infrequent presentation of these diseases in a young adult and the coexistence of two neoplasias characterized by typical chromosomal abnormalities, we consider the possibility of a genetic cancer susceptibility in our patient.

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Intramedullary Ewing sarcoma of the spinal cord: consequences of molecular diagnostics

Journal of Neurosurgery Spine ◽

10.3171/spi.2001.95.2.0270 ◽

2001 ◽

Vol 95 (2) ◽

pp. 270-275 ◽

Cited By ~ 4

Author(s):

Robert J. Weil ◽

Zhengping Zhuang ◽

Svetlana Pack ◽

Shimareet Kumar ◽

Lee Helman ◽

...

Keyword(s):

Spinal Cord ◽

Ewing Sarcoma ◽

Unusual Case ◽

Modern Medicine ◽

Round Cell ◽

Intramedullary Spinal Cord ◽

Content Type ◽

Small Round Cell ◽

Neuroectodermal Tumors ◽

Round Cell Tumors

✓ Molecular biological techniques have begun to transform modern medicine. These techniques have shown promise in the pathological diagnosis of difficult or uncommon tumors. Accurate molecular diagnosis of the small round-cell tumors, for example, is especially important because divergent therapies may be required to eradicate such disparate lesions as neuroblastoma, lymphoma, rhabdomyosarcoma, central primitive neuroectodermal tumors/medulloblastoma, or Ewing sarcoma (ES). The authors present an unusual case of a primary, extraosseous ES arising from the intramedullary spinal cord, in which molecular studies were required for specific diagnosis and therapeutic guidance.

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Primitive Neuroectodermal Tumor/Ewing Sarcoma Presenting with Pulmonary Nodular Lesions

Case Reports in Oncological Medicine ◽

10.1155/2015/957239 ◽

2015 ◽

Vol 2015 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Selvi Asker ◽

Fuat Sayir ◽

Gulay Bulut ◽

Aysel Sunnetcioglu ◽

Selami Ekin ◽

...

Keyword(s):

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Pulmonary Nodules ◽

Lung Parenchyma ◽

Round Cell ◽

X Ray ◽

Small Round Cell ◽

Neuroectodermal Tumors ◽

Chest X Ray ◽

Nodular Lesions

Primitive neuroectodermal tumors (PNETs) and Ewing sarcoma (EWS) belong to the same family of malignant, small, round cell neoplasms of soft tissue or bone origin. EWS-PNETs that arise in the lung parenchyma involvement are extremely rare in adults. A case of a 32-year-old male presenting with chest pain and diffuse pulmonary nodules on chest X-ray and diagnosed with Ewing sarcoma-PNETs will be presented here.

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Desmoplastic Small Round Cell Tumors: Cytologic, Histologic, and Immunohistochemical Features

Archives of Pathology & Laboratory Medicine ◽

10.5858/2006-130-728-dsrctc ◽

2006 ◽

Vol 130 (5) ◽

pp. 728-732 ◽

Cited By ~ 10

Author(s):

Fuju Chang

Keyword(s):

Differential Diagnosis ◽

Abdominal Cavity ◽

Cell Tumor ◽

Round Cell ◽

Cytologic Diagnosis ◽

Small Round Cell ◽

Karyotypic Abnormality ◽

The Family ◽

Cytogenetic Profile ◽

Round Cell Tumors

Abstract Desmoplastic small round cell tumor (DSRCT) is a recently recognized clinicopathologic entity that has a predilection for adolescent males and usually affects the abdominal cavity. Due to its uncommon nature, many pathologists lack experience with this tumor. The literature regarding DSRCT is reviewed with special attention to its histologic and cytologic diagnosis. Morphologic features of DSRCT and its immunohistochemical and cytogenetic profile are summarized and differential diagnosis with other small round cell tumors is discussed. As observed by both histologic and cytologic examinations, small round blue cells and fibrosclerotic stroma are the striking morphologic features of DSRCT. The typical immunohistochemical profile is characterized by coexpression of epithelial, mesenchymal, myogenic, and neural markers. Cytogenetically, this tumor harbors a specific karyotypic abnormality, namely t(11;22)(p13;q12). These features distinguish DSRCT from other members of the family of small round cell tumors.

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Extra-osseous Ewing sarcoma of the thyroid gland mimicking a hemorrhagic cyst of Hashimoto thyroiditis

Journal of Clinical Review & Case Reports ◽

10.33140/jcrc/02/01/00006 ◽

2017 ◽

Vol 2 (1) ◽

Keyword(s):

Ewing Sarcoma ◽

Hashimoto Thyroiditis ◽

Rare Entity ◽

Round Cell ◽

Ultrastructural Examination ◽

First Case ◽

Askin Tumor ◽

Neuroectodermal Tumors ◽

Ewing's Family Of Tumors ◽

Extraosseous Ewing Sarcoma

Ewing’s family of tumors, including Ewing sarcoma, Askin tumor, and peripheral primitive neuroectodermal tumors, were first identified by James Ewing in 1921. Ewing sarcoma is more common in bones. However, extraosseous Ewing sarcoma (EoES) is a very rare separate entity and was first described by Tefft and al. in 1969. It is a highly malignant round-cell tumor and contains a reciprocal translocation between chromosomes 11 and 22, t(11;22). In this report, we present the case of a 34 years old female patient who was unfortunately diagnosed with Ewing sarcoma of the thyroid after being operated for a hemorrhagic cyst in a context of Hashimoto thyroiditis.The patient was followed at the endocrinology department for an auto-immune thyroiditis for two years. She complained of neck pain and a suspicious hemorrhagic cyst was discovered on thyroid ultrasound. She underwent total thyroidectomy; three weeks later, she suffered from right sided firm swelling in her neck. The pathology was in favor of EoES of the thyroid. To date and to the best of our knowledge, this is the first case of Ewing sarcoma of the thyroid in auto-immune thyroiditis. EoES of the thyroid is a very rare entity and should be considered in the differential diagnosis of a solid tumor in the thyroid. Diagnosis is based on ultrastructural examination, immunostaining and evidence of abnormal translocation t(11;22). It is usually an aggressive disease.Early diagnosis with adequate surgical management and chemotherapy are the mainstay of the treatment and may change the outcome.

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Primary Pulmonary Primitive Neuroectodermal Tumor: A Case Report and Literature Review

10.52916/jmrs204032 ◽

2020 ◽

pp. 1-3

Author(s):

Guzmán-Casta Jordi

Keyword(s):

Case Report ◽

Soft Tissue ◽

Chest Wall ◽

Primitive Neuroectodermal Tumor ◽

Lung Parenchyma ◽

Round Cell ◽

Primitive Neuroectodermal Tumors ◽

Small Round Cell ◽

Molecular Features ◽

Neuroectodermal Tumors

Primitive Neuroectodermal Tumors (PNET) and Ewing Sarcoma (EWS) belong to the same family of malignant, small, round cell neoplasms of soft tissue or bone origin. Common locations for EWS-PNET include chest wall, pelvis, and extremities. EWSPNETs that arise in the lung parenchyma without chest wall involvement are extremely rare in adults. We report a case of EWS-PNET of the lung in an adult and review the distinct clinical, pathological, and molecular features of these tumors.

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