icd 10 code for desmoplastic small round cell tumor

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What is desmoplastic small round cell tumor?

Desmoplastic small round cell tumor (DSRCT) is a type of cancer. It is usually found in the abdomen (belly), but it can also occur in other parts of the body. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. The tumor has a specific genetic abnormality...

What is another name for desmoplastic sarcoma?

Alternative names. This disease is also known as: desmoplastic small round blue cell tumor; intra-abdominal desmoplastic small round blue cell tumor; desmoplastic small cell tumor; desmoplastic cancer; desmoplastic sarcoma; DSRCT.

What is a small round cell tumor called?

Also called: DSRCT, polyphenotypic small round cell tumor. Desmoplastic small round cell tumor (DSRCT) is a type of cancer. It is usually found in the abdomen (belly), but it can also occur in other parts of the body. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue.

What is a DSRCT tumor?

Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. The tumor has a specific genetic abnormality that helps confirm the diagnosis. DSRCT in the abdomen may not be found until the tumors have grown large. As a result, often the disease has spread to the liver, lymph nodes, lungs or bones by the time it is diagnosed.

What is a desmoplastic small round cell tumor?

Desmoplastic small round cell tumors, or DSRCT, are tumors that grow in the abdomen and pelvic area of the body. Desmoplastic small round cell tumors are a type of soft tissue sarcoma, which is a type of cancer that forms in the connective tissue of the body.

What is round cell tumor?

The term round cell tumor describes a group of highly aggressive malignant tumors composed of relatively small and monotonous undifferentiated cells with increased nuclear-cytoplasmic ratio.[1] Malignant small round cell tumors (MSRCT) is a term used for tumors composed of malignant round cells that are slightly larger ...

What is the ICD-10 code for desmoid tumor?

Right now, desmoid tumors fall under an ICD-10 code that covers a wide variety of connective and soft tissue tumors of “uncertain behavior”. Specifically, “D48. 1 – neoplasm of uncertain behavior of connective and other soft tissue.” This code encompasses dozens of different tumors.

What are the types of round cell tumors?

Round cell tumors fall into a few different categories: mast cell tumor, histiocytoma, lymphoma, plasmacytoma, and transmissible venereal tumors. Melanomas are the cytologic “great pretender” and, although classified as mesenchymal tumors, can appear as round cell tumors on cytology.

What is Desmoplastic?

Listen to pronunciation. (DES-moh-PLAS-tik) Causing or forming adhesions or fibrous connective tissue within a tumor.

What is desmoplastic stroma?

Desmoplasia is the result of increased synthesis of extracellular matrix proteins and collagen by stromal cells. It is considered to be a reaction and response of the host tissue against invasive cancer cells.[5]

What is a desmoid tumor?

Desmoid tumors are noncancerous growths that occur in the connective tissue. Desmoid tumors most often occur in the abdomen, arms and legs. Another term for desmoid tumors is aggressive fibromatosis. Some desmoid tumors are slow growing and don't require immediate treatment.

What is the ICD 10 code for retroperitoneal mass?

Malignant neoplasm of retroperitoneum C48. 0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2022 edition of ICD-10-CM C48. 0 became effective on October 1, 2021.

What is giant cell tumor of bone?

What is a giant cell tumor? Giant cell tumor of bone is a rare, aggressive non-cancerous tumor. It generally happens in adults between ages 20 and 40 when skeletal bone growth is complete. It usually develops near a joint at the end of the bone.

Is desmoplastic small round cell tumor curable?

Because DSRCT is so rare, no standard way to treat it has been developed. The following treatment methods have been used: Surgery — is used to remove as much of the cancer as possible. Often, DSRCTs have spread too far for complete removal, but surgeons try to remove at least 90 percent of them.

What do round cells mean?

Medical Definition of round cell : a small lymphocyte or a closely related cell especially occurring in an area of chronic infection or as the typical cell of some sarcomas.

Where are round cells found?

Round cell tumors are among the most common skin tumors in dogs, and they typically form just under the skin, although they may change the surface of the skin above them. Some round cell tumors are more worrisome than others.

Uncertain differentiation

Cite this page: Alexiev BA. Desmoplastic small round cell. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueDSRCT.html. Accessed December 7th, 2021.

Desmoplastic small round cell

Cite this page: Alexiev BA. Desmoplastic small round cell. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueDSRCT.html. Accessed December 7th, 2021.

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Epidemiology

DSRCT is extremely rare. Only a few hundred cases have been reported worldwide since the first description in 1989. It usually affects males, during adolescence or young adulthood, with a male-to-female ratio of 4:1.

Clinical description

Clinical signs and symptoms of DSRCT are non-specific. DSRCT presents with abdominal pain, enlarged abdomen, dyspepsia, and/or vomiting and weight loss depending on the stage of the disease. Other signs can be observed such as a palpable abdominal mass, gastrointestinal occlusion, ascites, and hepatomegaly.

Etiology

DSRCT seems to originate from the mesothelium. In almost all cases, a specific translocation t (11;22) (p13;q12) is found that juxtaposes the EWSR1 gene to the WT1 tumor suppressor gene. However, the underlying molecular mechanism remains unknown.

Diagnostic methods

The diagnosis is difficult due to the rarity of the tumor and its similarities with other small round cell tumors. Diagnosis is based on clinical signs, endoscopic examination (laparoscopy) and/or imaging techniques (radiography, chest-abdominal-pelvic computed tomography (CAP-CT)).

Differential diagnosis

Differential diagnoses include all the small round cells tumors: Ewing sarcoma and other peripheral neuroectodermal tumors (PNET), Wilms tumor, rhabdomyosarcoma and undifferentiated carcinoma (see these terms).

Management and treatment

Management is multidisciplinary and must be discussed by a panel of physicians in a specialized center. Up to 30% of DSRCT cases are misdiagnosed leading to incorrect management.

What is the code for a primary malignant neoplasm?

A primary malignant neoplasm that overlaps two or more contiguous (next to each other) sites should be classified to the subcategory/code .8 ('overlapping lesion'), unless the combination is specifically indexed elsewhere.

What chapter is neoplasms classified in?

All neoplasms are classified in this chapter, whether they are functionally active or not. An additional code from Chapter 4 may be used, to identify functional activity associated with any neoplasm. Morphology [Histology] Chapter 2 classifies neoplasms primarily by site (topography), with broad groupings for behavior, malignant, in situ, benign, ...

What is the table of neoplasms used for?

The Table of Neoplasms should be used to identify the correct topography code. In a few cases, such as for malignant melanoma and certain neuroendocrine tumors, the morphology (histologic type) is included in the category and codes. Primary malignant neoplasms overlapping site boundaries.

What is the code for a primary malignant neoplasm?

A primary malignant neoplasm that overlaps two or more contiguous (next to each other) sites should be classified to the subcategory/code .8 ('overlapping lesion'), unless the combination is specifically indexed elsewhere.

What is the table of neoplasms used for?

The Table of Neoplasms should be used to identify the correct topography code. In a few cases, such as for malignant melanoma and certain neuroendocrine tumors, the morphology (histologic type) is included in the category and codes. Primary malignant neoplasms overlapping site boundaries.

What is a desmoplastic small round cell tumor?

Desmoplastic small round cell tumor (DSRCT) is a rare type of soft tissue cancer ( sarcoma) that usually begins in the abdomen. It primarily affects children and young adults and is more common in males. It is formed by small, round cancer cells surrounded by scar-like tissue and is often found in the tissue (peritoneum) that lines the inside of the abdomen and pelvis. The tumor cells have a characteristic genetic change involving a translocation between chromosomes 11 and 22, which is important in differentiating from other similar tumors. The genetic change involved in DSRCT is acquired throughout a person's lifetime and is not inherited. [1] [2] [3]

Where is a tumor found?

It is formed by small, round cancer cells surrounded by scar-like tissue and is often found in the tissue (peritoneum) that lines the inside of the abdomen and pelvis. The tumor cells have a characteristic genetic change involving a translocation between chromosomes 11 and 22, which is important in differentiating from other similar tumors. ...

Is DSRCT inherited?

The genetic change involved in DSRCT is acquired throughout a person's lifetime and is not inherited. [1] [2] [3] Symptoms may include abdominal pain and a feeling of fullness, abdominal mass, and symptoms of gastrointestinal obstruction, such as constipation. Treatment options may include: chemotherapy, radiation therapy, surgery, ...

How to diagnose desmoplastic small round cell tumor?

What is the prognosis for someone with desmoplastic small round cell tumors? 1 Where the tumor is in your body 2 If the cancer has spread to other parts of your body 3 How much of the tumor was taken out during surgery

Where do desmoplastic small round cell tumors grow?

Desmoplastic small round cell tumors, or DSRCT, are tumors that grow in the abdomen and pelvic area of the body. Desmoplastic small round cell tumors are a type of soft tissue sarcoma, which is a type of cancer that forms in the connective tissue of the body. Connective tissues include fat, muscles, tendons, lymph and blood vessels, and nerves.

How to check if a tumor is DSRCT?

To check if the tumor is DSRCT your doctor will do a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope to see what kind of tumor it is. Under a microscope, DSRCT cells look round.

How rare is DSRCT?

DSRCT is very rare . DSRCT occurs most often in young white males between the ages of 10 and 30. Some reports say that only about 200 cases of DSRCT have been recorded since the cancer was first described in 1989.

What scans are used to detect DSRCT?

If you have symptoms of DSRCT, your doctor will use imaging scans such as ultrasound, CT, MRI, and PET, to look at where the tumors are and how big they are. They will also check for signs that the tumor has spread to other parts of the body.

What is a desmoplastic small round cell tumor?

Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, ...

What are the markers of tumor cells?

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.

How do you know if you have a tumor?

The tumors can be felt as hard, round masses by palpating the abdomen. First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and cachexia .

Is DSRCT a poor prognosis?

The prognosis for DSRCT remains poor. Prognosis depends upon the stage of the cancer. Because the disease can be misdiagnosed or remain undetected, tumors frequently grow large within the abdomen and metastasize or seed to other parts of the body.

Can a DSRCT be mistaken for a tumor?

DSRCT in young patients can be mistaken for other abdominal tumors including rhabdomyosarcoma, neuroblastoma, and mesenteric carcinoid.

Is a tumor a risk factor for childhood cancer?

There are no known risk factors that have been identified specific to the disease. The tumor appears to arise from the primitive cells of childhood, and is considered a childhood cancer.

Is DSRCT a misdiagnosed disease?

DSRCT is frequently misdiagnosed. Adult patients should always be referred to a sarcoma specialist. This is an aggressive, rare, fast spreading tumor and both pediatric and adult patients should be treated at a sarcoma center.

Where is a desmoplastic small round cell tumor found?

What is a desmoplastic small round cell tumor? Desmoplastic small round cell tumor (DSRCT) is a type of cancer. It is usually found in the abdomen (belly), but it can also occur in other parts of the body. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. The tumor has a specific genetic abnormality ...

What is the survival rate of desmoplastic small round cell tumor?

Currently DSRCT has a 15 percent five-year survival rate. However, researchers are looking for treatments that will improve those odds.

How rare is DSRCT?

DSRCT is extremely rare . Fewer than 200 cases have been reported, about 85 percent of them in Caucasian people. Boys and young men are about four times as likely to have DSRCT as girls and young women. It is usually diagnosed in males between ages 10 and 30.

What are the symptoms of a small round cell tumor?

What are the symptoms of desmoplastic small round cell tumor? Symptoms of desmoplastic small round cell tumor include: Pain or a lump in the abdomen. Cramping. Nausea. Vomiting. Diarrhea. Constipation. Trouble having a bowel movement and/or passing gas.

Can DSRCTs be removed?

Often, DSRCTs have spread too far for complete removal, but surgeons try to remove at least 90 percent of them. Hyperthermic intraperitoneal chemotherapy (HIPEC) — may be given during the surgery to kill cancer cells that cannot be removed surgically. (The patient also avoids the side effects of standard chemotherapy.)