TITLE

Desmoplastic small round cell tumour of childhood: a report of four cases demonstrating wider clinical features and variable outcome

AUTHOR(S)
Ray, Ruma; Traunecker, Heidi C. L.; Raafat, Faro; Stevens, Michael C. G.
PUB. DATE
June 1997
SOURCE
Sarcoma;Jun1997, Vol. 1 Issue 2, p103
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Purpose. Four further cases of desmoplastic small round cell tumour with multi phenotypic differentiation are described. Subjects. Two patients were typical (male, adolescent with peritoneal tumour and, in one case, liver metastases) and did not respond well to treatment. Two other patients showed less usual features (young, female with retroperitoneal disease, both with intraspinal extension and renal tract obstruction). Both responded favourably to multi-modal treatment regimens including extensive and invasive supportive care. Results. Histologically, all tumours showed clear features of this diagnosis, namely angulated nests of small cells in a background of fibrovascular stroma. Immunohistochemistry typically showed divergent differentiation with neural, muscle and epithelial marker positivity. All four tumours stained positive for the Wilms' tumour 1 suppressor gene product. Electron microscopy showed intercellular tight junctions, cytoplasmatic intermediate filaments and absence of microvilli. Rare neurosecretory-type granules were observed. Discussion. These cases illustrate a broader spectrum of clinical presentation than previously associated with this diagnosis.
ACCESSION #
7620828

 

Related Articles

  • Immunohistochemical classification of B cell neoplasms. Oudejans, J.J.; van der Valk, P. // Journal of Clinical Pathology;Mar2003, Vol. 56 Issue 3, p193 

    Presents a table that illustrates the immunohistochemical classification of B cell neoplasms. Depiction of the most discriminating markers in relation to the most frequently occurring entities.

  • A Pelvic Mass in a 20-Year-Old Man. Sun-Young Jun; Jooryung Huh, Sung-Hye; Park, Jae Y.; Ro // Archives of Pathology & Laboratory Medicine;Oct2003, Vol. 127 Issue 10, pe411 

    Presents the case of a 20-year-old Korean man diagnosed with diffuse large B-cell lymphoma with Homer-Wright-type Rosette formation. Medical history of the patient; Results of examination by light microscopy; Immunohistochemistry technique for tumor diagnosis; Considerations in the diagnosis of...

  • Malignant Granular Cell Tumor of Soft Tissues: A Study of Two New Cases. Cruz-Mojarrieta, Julia; Navarro, Samuel; Gomez-Cabrera, Erasmo; Perez-Pena, Lourdes; Soriano, Pilar; Peydro-Oyala, Amando; Llombart-Bosch, Antonio // International Journal of Surgical Pathology;Jul2001, Vol. 9 Issue 3, p255 

    Examines the malignant granular cell tumor of soft tissues in Spain. Detection of high expression of p53 and MiB1 (Ki67); Morphologic and immunohistochemical criteria of malignancy of granular cell tumors; Cytologic features of tumoral cells.

  • Immunohistochemically identified double-hit lymphoma associated with poor outcomes.  // Hem/Onc Today;8/10/2012, Vol. 13 Issue 15, p40 

    The article discusses the results of a study which revealed the effectiveness of using immunohistochemical analysis in identifying the subset of patients with diffuse large B-cell lymphoma who have double-hit lymphomas compared to fluorescent in situ hybridization.

  • Trichogerminoma, a rare cutaneous follicular neoplasm with indolent clinical course: report of two cases and review of literature. Li-li Chen; Jin-tao Hu; Yang Li // Diagnostic Pathology;2013, Vol. 8 Issue 1, p1 

    Trichogerminoma is a rare cutaneous adnexal neoplasm of the hair germ cell and usually associated with benign clinical course and favorable outcome. Since its first description by Sau et al. in 1992, only a few cases have been reported up to date. Herein, we report two additional cases occurring...

  • Primary Vulval Rhabdoid Tumor in an Adult: A Case Report, Immunohistochemical Profile and Literature Review. Arafah, Maria A.; Aljuboury, Muna I. // Case Reports in Medicine;2011, p1 

    We report a rare case of primary vulval rhabdoid tumor in an adult. The diagnosis was confirmed using the recently emerging INI1/BAF47 immunostain.We also demonstrate the expression of ER and PR hormonal receptors by the tumor cells.

  • Use of immunohistochemistry in the differential diagnosis of cutaneous round cell tumours in dogs. Araújo, Marina R.; Preis, Ingred S.; Lavale, Gleidice E.; Cassali, Geovanni D.; Ecco, Roselene // BMC Proceedings;2013, Vol. 7 Issue Suppl 2, p1 

    The article presents information on a study related to the use of immunohistochemistry in the diagnosis of cutaneous round cell tumours in dogs. The method includes diagnosis of neoplastic tumours by histology and histochemical staining. The results showed modification of histologic diagnosis in...

  • Intravascular Large B-Cell Lymphoma. Orwat, Dennis E.; Batalis, Nicholas I. // Archives of Pathology & Laboratory Medicine;Mar2012, Vol. 136 Issue 3, p333 

    A rare type of diffuse large B-cell lymphoma, intravascular large B-cell lymphoma primarily affects the middleaged to elderly population, with a slight predominance in men. By the time of presentation, most patients have advanced, disseminated disease, and often the diagnosis is made at autopsy....

  • Primary Cutaneous Diffuse Large B.Cell Lymphoma of the Upper Limb: A Fascinating Entity. Gopal, Manoj Madakshira; Malik, Ajay // Indian Journal of Dermatology;Sep/Oct2013, Vol. 58 Issue 5, p366 

    Primary cutaneous lymphomas are defined as lymphoid neoplasms that present themselves clinically on the skin and do not have extra‑cutaneous disease, when the diagnosis is made or even after 6 months of the diagnosis. Primary cutaneous lymphomas of B‑cells are less frequent than...

Share

Read the Article

Courtesy of your local library

Public Libraries Near You (See All)
Looking for a Different Library?

Other Topics