Discuss This Disease

4 free views remaining today

esophageal adenoid cystic carcinoma

Description

Signs and Symptoms

Common Signs and Symptoms of Esophageal Adenoid Cystic Carcinoma

Esophageal adenoid cystic carcinoma (EACC) is a rare form of cancer that affects the esophagus. The symptoms of EACC can vary from person to person, but here are some common signs and symptoms associated with this condition:

  • Progressive dysphagia: This is the most common symptom of EACC, where patients experience difficulty swallowing food, which can lead to weight loss and malnutrition.
  • Upper abdominal pain or discomfort: Patients may experience pain or discomfort in the upper abdomen, especially after meals.
  • Heartburn and retrosternal pain: Some patients may experience heartburn and pain in the chest area.
  • Weight loss: As the cancer progresses, patients may experience weight loss due to difficulty swallowing and eating.

Other Possible Symptoms

In some cases, EACC can also cause other symptoms such as:

  • Painless lump or swelling: A painless lump or swelling in the esophagus or surrounding tissues.
  • Facial numbness and weakness: In rare cases, EACC can spread to the nerves in the face, causing numbness and weakness.

Diagnosis

The diagnosis of EACC is typically made through a combination of:

  • Endoscopy: A procedure where a flexible tube with a camera is inserted into the esophagus to visualize the tumor.
  • PET-CT scan: A imaging test that uses a combination of positron emission tomography (PET) and computed tomography (CT) scans to stage the cancer.
  • Endoscopic ultrasound: A procedure that uses high-frequency sound waves to create images of the esophagus and surrounding tissues.

It's essential to seek medical attention if you or someone you know is experiencing any of these symptoms. Early detection and treatment can improve outcomes for patients with EACC.

References

[3] The main symptoms associated with EACC include upper abdominal pain or discomfort following meals and progressive dysphagia. Common endoscopic findings include [4]. [5] The main symptoms associated with EACC include upper abdominal pain or discomfort following meals and progressive dysphagia. Common endoscopic findings include [6] Patients may be asymptomatic or may present with progressive dysphagia, heartburn, retrosternal pain and/or weight loss. [7] Symptoms are progressive dysphagia and weight loss. Diagnosis is by endoscopy, followed by PET-CT and endoscopic ultrasound for staging.

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Esophageal Adenoid Cystic Carcinoma

Esophageal adenoid cystic carcinoma (EACC) is a rare and malignant tumor that requires accurate diagnosis to ensure effective treatment. The following diagnostic tests are commonly used to diagnose EACC:

  • Endoscopy with cytology and biopsy: This is the primary diagnostic test for EACC, which involves inserting a flexible tube with a camera and light on the end (endoscope) into the esophagus to visualize the tumor. A small sample of tissue (biopsy) is then taken from the tumor for histopathological examination.
  • Barium x-ray procedures: These imaging tests may be used to show the location and size of the tumor in the esophagus. However, they are not as accurate as endoscopy with cytology and biopsy.
  • Gastrointestinal endoscopic ultrasound fine-needle aspiration: This test uses a specialized endoscope with an ultrasound probe to visualize the tumor and take a sample of tissue for examination.

Diagnostic Challenges

The diagnosis of EACC can be challenging due to its rarity and similarity in appearance to other esophageal tumors. A low diagnostic yield has been reported in the literature, highlighting the need for accurate and reliable diagnostic tests.

  • Symptoms and physical findings: The symptoms and physical findings of EACC may not always be characteristic, making it difficult to diagnose based on clinical evaluation alone.
  • Imaging and endoscopy: Imaging tests such as barium x-ray procedures and endoscopy with cytology and biopsy are essential for accurate diagnosis.

References

[1] Mediastinum & Esophagus: Case Report Two Roads to Diagnose Primary Esophageal Adenoid Cystic Carcinoma in the Mediastinum Pedro Juan Baez, MD,1 Mariana Mercader-Perez, MD,1 [2] Diagnostic Tests for Esophageal Adenoid Cystic Carcinoma: A Review of Current Literature.

Treatment

Differential Diagnosis

Differential Diagnosis of Esophageal Adenoid Cystic Carcinoma

Esophageal adenoid cystic carcinoma (EACC) is a rare and malignant tumor that can be challenging to diagnose. The differential diagnosis for EACC includes several conditions that may present with similar symptoms and imaging findings.

  • Squamous Cell Carcinoma (SCC): SCC is the most common type of esophageal cancer, and it can be difficult to distinguish from EACC based on clinical manifestations and imaging examinations alone [2]. However, EACC typically lacks squamous cells, central necrosis, or prominent mitotic images [10].
  • Basaloid Squamous Carcinoma: This is another type of esophageal cancer that may be considered in the differential diagnosis for EACC due to its pseudoglandular pattern akin to ACC. However, basaloid SCC typically contains squamous cells and has a more aggressive clinical course [10].
  • Metastasis or Direct Extension from Tumors of Lung or Breast: In some cases, EACC may be mistaken for metastatic disease or direct extension from tumors in the lung or breast due to its rarity and similar morphological features [8].

Key Features to Distinguish EACC from Other Conditions

To accurately diagnose EACC, it is essential to consider the following key features:

  • Histologic Appearance: EACC typically presents with a cribriform or tubular pattern of basaloid cells with small, angulated, and hyperchromatic nuclei and scant cytoplasm [14].
  • Imaging Findings: EACC may present with progressive dysphagia and food regurgitation on imaging studies, but these findings can be non-specific and require careful interpretation in the context of clinical symptoms and histologic features.
  • Clinical Presentation: The most common clinical presentations include progressive dysphagia and food regurgitation, which can be similar to those seen in other esophageal cancers.

Conclusion

In conclusion, differential diagnosis for EACC is a complex process that requires careful consideration of clinical manifestations, imaging findings, and histologic features. Accurate diagnosis is essential to provide appropriate treatment and improve patient outcomes.

References:

[2] - The clinical manifestations and imaging examinations of EACC often lack specificity, leading to potential misdiagnosis as esophageal SCC and other conditions. [8] - Differential diagnosis includes metastasis or direct extension from tumors of lung or breast. Morphologically these mimic primary adenocarcinoma ... [10] - Basal-like SCC may be considered in differential diagnosis due to its pseudoglandular pattern akin to ACC. However, ACC typically lacks squamous cells, central necrosis, or prominent mitotic images [14] - Abstract. Adenoid cystic carcinoma is a malignant tumor with a deceptively benign histologic appearance characterized by indolent, locally invasive growth with high propensity for local recurrence and distant metastasis.

Additional Information

oboInOwl#hasOBONamespace
disease_ontology
oboInOwl#id
DOID:4878
core#notation
DOID:4878
oboInOwl#hasDbXref
UMLS_CUI:C1333441
oboInOwl#hasExactSynonym
adenoid cystic carcinoma, esophagus
IAO_0000115
An esophageal carcinoma that derives_from epithelial cells of the salivary gland.
rdf-schema#label
esophageal adenoid cystic carcinoma
oboInOwl#inSubset
http://purl.obolibrary.org/obo/doid#NCIthesaurus
rdf-schema#subClassOf
http://purl.obolibrary.org/obo/DOID_1107
22-rdf-syntax-ns#type
http://www.w3.org/2002/07/owl#Class
rdf-schema#domain
https://w3id.org/def/predibionto#has_symptom_11184
owl#annotatedSource
t432794

Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.

It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.