Find information on thousands of medical conditions and prescription drugs.

Desmoplastic small round cell tumor

Desmoplastic small round cell tumor is classified as a soft tissue sarcoma. It is an aggressive and rare tumor that primarily occurs as multiple masses in the abdomen. Other areas affected include the lymph nodes, the lining of the abdomen, diaphragm , and the pelvis. The most common site of metatastic spred is to the liver, lungs, and bones. more...

Home
Diseases
A
B
C
D
Dandy-Walker syndrome
Darier's disease
Dementophobia
Demyelinating disease
Dendrophobia
Dengue fever
Dental fluorosis
Dentinogenesis imperfecta
Dentophobia
Depersonalization disorder
Dermatitis herpetiformis
Dermatofibroma
Dermatographic urticaria
Dermatomyositis
Dermatophytosis
Desmoplastic small round...
Dextrocardia
Diabetes insipidus
Diabetes mellitus
Diabetes, insulin dependent
Diabetic angiopathy
Diabetic nephropathy
Diabetic neuropathy
Diamond Blackfan disease
Diastrophic dysplasia
Dibasic aminoaciduria 2
Diethylstilbestrol...
DiGeorge syndrome
Dilated cardiomyopathy
Diphallia
Diphtheria
Dipsophobia
Dissociative amnesia
Dissociative fugue
Dissociative identity...
Distemper
Diverticulitis
Diverticulosis
Dk phocomelia syndrome
Doraphobia
Double outlet right...
Downs Syndrome
Dracunculiasis
Duane syndrome
Dubin-Johnson syndrome
Dubowitz syndrome
Duchenne muscular dystrophy
Dupuytren's contracture
Dwarfism
Dysbarism
Dysgerminoma
Dyskeratosis congenita
Dyskinesia
Dysmorphophobia
Dysplasia
Dysplastic nevus syndrome
Dysthymia
Dystonia
E
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Medicines

The tumor predominately strikes teenagers and young adults. It usually affects males, more than females.

There is no known risk factors that have been identified specific to the disease. Research has indicated that there is a relationship between desmoplastic small round cell tumor and the Ewings Family of Tumors.

DSRCT is associated with a unique chromosomal translocation (t11;22)(p13:q12) resulting in a chimeric EWS/WTI transcript that is diagnostic of this tumor. This transcript codes for a protein that acts as a transcriptional activator that fails to suppress tumor growth.

Symtoms of disease include abdominal pain, abdominal mass, gastrointestinal obstruction, ascites, possible anemia, and cachexia.

Pathology reveals well circumscribed solid tumor nodules within a dense desmoplastic stroma. Often areas of central necrosis are present. Tumor cells have hyperchromatic nuclei with increased nuclear/cytoplasmic ratio.

On immunohistochemistry, these cells have trilinear coexpression including the epithelial marker cytokeratin, the mesenchymal markers desmin and vimentin, and the neuronal marker neuron-specific enolase. Thus, although initially thought to be of mesothelial origin due to sites of presentation, it is now hypothesized to arise from a progenitor cell with multiphenotypic differentiation.

At this time, the prognosis for desmoplastic small round cell tumor is poor, less than 20%. Usually, by the time the disease is diagnosed the tumor has already grown large within the abdomen and metastasized or seeded to other parts of the body.

Although the tumors are described as chemo-resistant, there is treatment for the disease. Recent journals have reported that some patients respond to high dose chemotherapy, debulking operation , and radiation therapy. Other treatment options considered include: Autologous Stem cell Transplant, Intensity-Modulated Radiation Therapy, Stereotactic Body radiation therapy, and Intraperitoneal hyperthermic chemoperfusion.

This disease is also known as: Desmoplastic small round blue cell tumor; Intraabdominal desmoplastic small round blue cell tumor; Desmoplastic small cell tumor; Desmoplastic sarcoma; DSRCT.

Read more at Wikipedia.org


[List your site here Free!]



Desmoplastic small round cell tumor of the lung
To the Editor-I read with interest the recent article entitled "Desmoplastic Small Round Cell Tumor of the Lung" by Syed et al.1 The authors thoroughly ...
Desmoplastic small round cell tumor of the lung
An extra-abdominal desmoplastic small round cell tumor (DSRCT) of the lung with immunohistochemical, ultrastructural, and cytogenetic evidence of multidirectional ...
Giant Cell Tumor of Rib Masquerading as Thymoma: A Diagnostic Pitfall in Needle Core Biopsy of the Mediastinum
Giant cell tumor of bone is rarely seen in the rib, where it may present as a mediastinal mass. The diagnosis of giant cell tumor of bone is generally ...
Primary Small Bowel Carcinoid Tumor With Bilateral Breast Metastases: Report of 2 Cases With Different Clinical Presentations
Context.-Carcinoid tumor metastatic to the breast is uncommon and can closely mimic a mammary carcinoma. The differentiation of metastatic carcinoid tumor ...
Clear cell carcinoid tumor of the gallbladder: A case without von Hippel-Lindau disease
A golden yellow polyp was detected in the gallbladder of a 64-year-old man who presented with epigastric pain. The lesion was composed of clear polygonal ...
Primitive Neuroectodermal Tumor of the Stomach
Ewing sarcoma/primitive neuroectodermal tumor is classically a tumor of the soft tissue or bone in children and young adults, but several cases have been ...
Immunohistochemical Expression of Wilms Tumor Gene Protein in Different Histologic Subtypes of Ovarian Carcinomas
Context.-Immunohistochemical expression of Wilms tumor gene protein (WT1) has previously been described in primary ovarian carcinomas. Objective.-To evaluate ...
Pathologic Quiz Case: A 79-Year-Old Woman With a Black, Ulcerated Cecal Tumor and 3 Negative Guaiac Test Results
A 79-year-old woman complained of increasing fatigue with a few episodes of dizziness. Past medical history included hypertension, total hysterectomy at the age of 37 years for a fibroid uterus, and

Home Contact Resources Exchange Links ebay