ICD-10: D48.114

Desmoid tumor, intraabdominal

Clinical Information

Inclusion Terms

  • Desmoid tumor, peritoneal, retroperitoneal
  • Desmoid tumor of pelvic cavity

Additional Information

Clinical Information

Desmoid tumors, also known as aggressive fibromatosis, are benign but locally aggressive neoplasms that can arise in various locations, including the intraabdominal region. The clinical presentation, signs, symptoms, and patient characteristics associated with desmoid tumors, particularly those coded as ICD-10 D48.114 (Desmoid tumor, intraabdominal), are essential for accurate diagnosis and management.

Clinical Presentation

Desmoid tumors typically present as painless masses, but their clinical manifestations can vary significantly based on their size, location, and the structures they involve. Intraabdominal desmoid tumors may arise from the abdominal wall, mesentery, or retroperitoneum, leading to a range of symptoms.

Signs and Symptoms

  1. Abdominal Pain: Patients may experience vague or localized abdominal pain, which can be attributed to the tumor's pressure on surrounding structures or organs[1].

  2. Palpable Mass: In some cases, a palpable mass may be detected during a physical examination, particularly if the tumor is large enough to be felt through the abdominal wall[1].

  3. Bowel Obstruction: Due to their location, intraabdominal desmoid tumors can cause bowel obstruction, leading to symptoms such as nausea, vomiting, and changes in bowel habits[1][2].

  4. Weight Loss: Unintentional weight loss may occur, often related to pain or discomfort associated with eating[2].

  5. Ascites: In some instances, patients may develop ascites, which is the accumulation of fluid in the abdominal cavity, potentially due to tumor-related complications[2].

  6. Other Symptoms: Depending on the tumor's location, patients may also experience urinary symptoms or other organ-specific issues if the tumor compresses adjacent structures[1].

Patient Characteristics

Desmoid tumors can occur in a wide range of patients, but certain characteristics and risk factors are noteworthy:

  1. Age and Gender: Desmoid tumors are most commonly diagnosed in young adults, particularly those aged 15 to 60 years. They have a slight female predominance[1][2].

  2. Genetic Factors: Patients with familial adenomatous polyposis (FAP) are at a significantly increased risk for developing desmoid tumors. This genetic condition predisposes individuals to various types of tumors, including desmoids, particularly after surgical procedures like colectomy[2].

  3. History of Trauma or Surgery: There is often a correlation between the development of desmoid tumors and a history of trauma or surgical intervention in the affected area, which may trigger tumor growth[1].

  4. Hormonal Influences: Some studies suggest that hormonal factors may play a role in the development of desmoid tumors, as they can be influenced by estrogen levels, particularly in women[2].

  5. Location-Specific Characteristics: Intraabdominal desmoid tumors may have specific characteristics based on their anatomical location, affecting their clinical behavior and management strategies[1].

Conclusion

Desmoid tumors, particularly those classified under ICD-10 code D48.114, present a unique clinical challenge due to their benign nature yet aggressive local behavior. Understanding the signs, symptoms, and patient characteristics associated with intraabdominal desmoid tumors is crucial for timely diagnosis and effective management. Clinicians should maintain a high index of suspicion, especially in patients with relevant risk factors, to ensure appropriate intervention and monitoring.

Approximate Synonyms

Desmoid tumors, particularly those classified under the ICD-10 code D48.114, which refers specifically to intraabdominal desmoid tumors, are known by various alternative names and related terms. Understanding these terms can be crucial for accurate diagnosis, treatment, and coding in medical settings.

Alternative Names for Desmoid Tumors

  1. Desmoid Fibroma: This term emphasizes the fibrous nature of the tumor, highlighting its composition.
  2. Aggressive Fibromatosis: This name reflects the tumor's tendency to invade surrounding tissues aggressively, despite being benign.
  3. Desmoid-Type Fibromatosis: This term is often used interchangeably with desmoid tumors, particularly in clinical and research contexts.
  4. Intraabdominal Fibromatosis: Specifically refers to desmoid tumors located within the abdominal cavity, aligning closely with the D48.114 classification.
  1. Benign Soft Tissue Tumor: Desmoid tumors are classified as benign, meaning they do not metastasize like malignant tumors, although they can be locally aggressive.
  2. Fibrous Tumor: This broader category includes various tumors composed of fibrous tissue, of which desmoid tumors are a specific type.
  3. Extra-abdominal Desmoid Tumor: While D48.114 specifically refers to intraabdominal tumors, this term is relevant for understanding the broader category of desmoid tumors that can occur outside the abdominal cavity.
  4. Familial Adenomatous Polyposis (FAP): This genetic condition is associated with a higher risk of developing desmoid tumors, particularly in the abdominal region.

Clinical Context

Desmoid tumors are often linked to trauma, surgery, or genetic predispositions, such as FAP. They can occur in various locations, including the abdomen, and their management may involve surgical intervention, observation, or other therapeutic approaches depending on their size and symptoms.

Understanding these alternative names and related terms is essential for healthcare professionals involved in the diagnosis and treatment of desmoid tumors, as well as for accurate coding and billing practices in medical records.

Diagnostic Criteria

Diagnosing a desmoid tumor, particularly one classified under ICD-10 code D48.114 (desmoid tumor, intraabdominal), involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below is a detailed overview of the criteria and processes typically used in the diagnosis of intraabdominal desmoid tumors.

Clinical Evaluation

Symptoms

Patients may present with a variety of symptoms, which can include:
- Abdominal pain or discomfort
- A palpable mass in the abdomen
- Changes in bowel habits, depending on the tumor's location
- Symptoms related to compression of adjacent structures, such as urinary or gastrointestinal issues

Medical History

A thorough medical history is essential, particularly looking for:
- Previous surgeries, especially in the abdominal area, as desmoid tumors can arise post-operatively (post-surgical desmoid tumors).
- Family history of familial adenomatous polyposis (FAP) or other genetic syndromes associated with desmoid tumors.

Imaging Studies

Ultrasound

  • An initial ultrasound may be performed to assess the presence of a mass and its characteristics, such as size and location.

CT Scan or MRI

  • CT Scan: A computed tomography (CT) scan of the abdomen is often used to provide detailed images of the tumor, its size, and its relationship to surrounding structures.
  • MRI: Magnetic resonance imaging (MRI) can be particularly useful for evaluating soft tissue characteristics and determining the extent of the tumor.

Histopathological Examination

Biopsy

  • A definitive diagnosis typically requires a biopsy. This can be performed via:
  • Core needle biopsy: A minimally invasive procedure to obtain tissue samples.
  • Excisional biopsy: In some cases, the entire tumor may be removed for examination.

Pathological Analysis

  • The biopsy sample is examined microscopically to confirm the diagnosis. Desmoid tumors are characterized by:
  • Fibroblastic proliferation
  • Lack of significant cellular atypia
  • A desmoplastic stroma

Differential Diagnosis

It is crucial to differentiate desmoid tumors from other abdominal masses, such as:
- Sarcomas
- Gastrointestinal stromal tumors (GISTs)
- Other soft tissue tumors

Genetic Testing

In cases where familial syndromes are suspected, genetic testing may be recommended to identify mutations associated with conditions like FAP, which can predispose individuals to desmoid tumors.

Conclusion

The diagnosis of an intraabdominal desmoid tumor (ICD-10 code D48.114) is a multifaceted process that combines clinical assessment, imaging studies, and histopathological evaluation. Given the complexity and potential for misdiagnosis, a multidisciplinary approach involving oncologists, radiologists, and pathologists is often beneficial to ensure accurate diagnosis and appropriate management of the condition.

Treatment Guidelines

Desmoid tumors, classified under ICD-10 code D48.114, are benign but aggressive fibromatosis that can occur in various locations, including intra-abdominal regions. These tumors are characterized by their tendency to infiltrate surrounding tissues and can cause significant morbidity due to their local effects. The management of desmoid tumors, particularly those located intra-abdominally, involves a multidisciplinary approach that may include observation, surgical intervention, and adjuvant therapies.

Standard Treatment Approaches

1. Observation

For asymptomatic desmoid tumors or those that are small and not causing any significant issues, a watchful waiting approach may be adopted. Regular imaging and clinical evaluations are conducted to monitor tumor growth. This strategy is particularly relevant given that some desmoid tumors can stabilize or even regress over time without intervention[1].

2. Surgical Resection

Surgical excision is often considered the primary treatment for symptomatic desmoid tumors or those that are growing. The goal of surgery is to achieve complete resection with clear margins, which can significantly reduce the risk of recurrence. However, due to the infiltrative nature of these tumors, achieving clear margins can be challenging, and recurrence rates can be high even after complete resection[1][2].

3. Radiation Therapy

In cases where surgical resection is not feasible or if the tumor is unresectable, radiation therapy may be employed. Intensity-modulated radiation therapy (IMRT) is one of the advanced techniques used to target the tumor while minimizing damage to surrounding healthy tissues. Radiation therapy can be particularly beneficial for patients who are not surgical candidates or for those with recurrent disease[2][3].

4. Medical Therapy

For patients with progressive or symptomatic desmoid tumors who are not candidates for surgery or radiation, medical management may be considered. This can include:
- Nonsteroidal Anti-Inflammatory Drugs (NSAIDs): These can help manage pain and may have a role in tumor control.
- Hormonal Therapy: Agents such as tamoxifen or aromatase inhibitors have been used, particularly in cases where hormonal influences are suspected to play a role in tumor growth.
- Targeted Therapies: Newer agents, such as tyrosine kinase inhibitors (e.g., imatinib), are being investigated for their efficacy in treating desmoid tumors, although their use is still largely experimental[1][3].

5. Clinical Trials

Given the complex nature of desmoid tumors and the variability in treatment response, participation in clinical trials may be an option for some patients. These trials can provide access to novel therapies and contribute to the understanding of effective treatment strategies for desmoid tumors[1].

Conclusion

The management of intra-abdominal desmoid tumors (ICD-10 code D48.114) requires a tailored approach based on the tumor's characteristics, the patient's symptoms, and overall health. While observation may be appropriate for some, surgical resection remains a cornerstone of treatment for symptomatic cases. Radiation therapy and medical management provide additional options, particularly for patients who are not surgical candidates. Ongoing research and clinical trials continue to explore new treatment modalities, offering hope for improved outcomes in managing this challenging condition.

For any specific treatment decisions, it is essential for patients to consult with a multidisciplinary team experienced in managing desmoid tumors to determine the most appropriate course of action based on individual circumstances[2][3].

Description

Desmoid tumors, also known as aggressive fibromatosis, are benign but locally aggressive tumors that arise from connective tissue. They can occur in various locations, including the abdominal cavity, which is classified under the ICD-10 code D48.114. Below is a detailed clinical description and relevant information regarding this specific code.

Clinical Description of Desmoid Tumors

Definition and Characteristics

Desmoid tumors are characterized by their fibrous tissue composition and can be classified as soft tissue tumors. Although they are not malignant, they can exhibit aggressive behavior, leading to local invasion and recurrence. These tumors are often associated with conditions such as familial adenomatous polyposis (FAP) and can occur sporadically.

Intraabdominal Location

When desmoid tumors occur in the intraabdominal region, they may arise from the abdominal wall, mesentery, or retroperitoneum. The intraabdominal location can complicate diagnosis and treatment due to the proximity to vital organs and structures.

Symptoms

Patients with intraabdominal desmoid tumors may present with a variety of symptoms, including:
- Abdominal pain or discomfort
- Palpable mass in the abdomen
- Bowel obstruction or changes in bowel habits
- Weight loss or anorexia in advanced cases

Diagnosis

Diagnosis typically involves imaging studies such as ultrasound, CT scans, or MRI to assess the tumor's size, location, and relationship to surrounding structures. A biopsy may be performed to confirm the diagnosis histologically.

Treatment Options

Treatment for desmoid tumors can vary based on the tumor's size, location, and symptoms. Options include:
- Surgical Resection: The primary treatment for localized tumors, aiming for complete removal.
- Observation: In asymptomatic cases or when surgery poses significant risks, a watchful waiting approach may be adopted.
- Medical Therapy: Options such as nonsteroidal anti-inflammatory drugs (NSAIDs), hormonal therapy, or targeted therapies may be considered, especially for unresectable tumors or those that recur.

ICD-10 Code D48.114

Code Details

  • ICD-10 Code: D48.114
  • Description: Desmoid tumor, intraabdominal
  • Category: This code falls under the category of "Neoplasms of uncertain behavior," indicating that while the tumor is not malignant, it has the potential for aggressive local behavior.

Importance of Accurate Coding

Accurate coding is crucial for proper documentation, treatment planning, and reimbursement. The use of D48.114 helps healthcare providers communicate the specific nature of the tumor, which is essential for clinical management and research purposes.

Conclusion

Desmoid tumors, particularly those located intraabdominally, present unique challenges in diagnosis and management. The ICD-10 code D48.114 serves as a critical tool for healthcare professionals in categorizing and treating these tumors effectively. Understanding the clinical characteristics, symptoms, and treatment options is essential for optimal patient care and outcomes.

Related Information

Clinical Information

  • Painless masses typically present
  • Abdominal pain can occur due pressure
  • Palpable mass may be detected physically
  • Bowel obstruction possible due location
  • Weight loss may result from pain or discomfort
  • Ascites can develop due to complications
  • Young adults predominantly affected gender-wise
  • FAP patients at increased risk genetically
  • Trauma or surgery often precedes development
  • Hormonal influences may play a role

Approximate Synonyms

  • Desmoid Fibroma
  • Aggressive Fibromatosis
  • Desmoid-Type Fibromatosis
  • Intraabdominal Fibromatosis
  • Benign Soft Tissue Tumor
  • Fibrous Tumor
  • Extra-abdominal Desmoid Tumor

Diagnostic Criteria

  • Clinical evaluation with thorough medical history
  • Abdominal pain or discomfort as primary symptom
  • Palpable abdominal mass present in most cases
  • Imaging studies, including ultrasound, CT scan, and MRI
  • Histopathological examination via biopsy for diagnosis
  • Fibroblastic proliferation characteristic of desmoid tumors
  • Lack of cellular atypia in histopathology

Treatment Guidelines

  • Surgical excision with clear margins
  • Observation for small asymptomatic tumors
  • Radiation therapy for unresectable tumors
  • Medical management with NSAIDs and hormonal therapy
  • Targeted therapies like tyrosine kinase inhibitors
  • Clinical trials for novel treatments
  • Multidisciplinary approach to treatment planning

Description

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