Adenoid Cystic Carcinoma
Description
● Introduction:
A uncommon but aggressive cancer of the small salivary glands of the palate, adenoid cystic carcinoma (ACC) is characterised by sluggish development, frequent invasion of the perineural space, and a high risk of distant metastases and local recurrence. The solid type is the most aggressive and linked to the poorest prognosis. It displays three different histological patterns: cribriform, tubular, and solid. Even if initial survival rates are high, the tumor's tendency towards late metastasis might have a negative impact on long-term results.
● Case presentation:
• The patient, a 46-year-old man who smokes, complained of pain and •swelling in the vicinity of his left upper molar (tooth 26).
➣Results:
• Palatal oedema in Caries 26 (20.5 × 17.1 mm).
• Sturdy, nontender, smooth, and immovable bulk.
• Cribriform ADCC (no perineural invasion) is the result of biopsy.
➣ Treatment: Post-operative radiation + left hemimaxillectomy + wide excision.
➣ Stage: T3N0M0 (AJCC).
➣ Regular follow-up is necessary because of the high risk of recurrence.
● The study of epidemiology:
• Approximately 10% of salivary gland tumours are rare.
• ~1% of all cancers of the head and neck.
• The most prevalent malignant tumour of the small salivary glands and submandibular region.
➣Sites: • Parotid (most common main gland).
• In 50% of intraoral instances, the palate is hard.
• Other areas include the floor of the mouth, nose, sinuses, lips, retromolar, sublingual gland, and buccal mucosa.
● Clinical Characteristics:
➣Pattern of growth: recurring, persistent, and indolent.
➣ Presentation:
• Swelling that is firm and painless, which is frequently confused with a benign lesion.
• If there is a perineural invasion, it might be painful.
➣ Late Metastasis: Liver, bones, and lungs Rarely implicated are regional lymph nodes.
● Histology
➣ Subtypes:
• Tubular: best prognosis, clearly distinguished.
• Cribriform: "Swiss-cheese" pattern; prognosis is intermediate.
• Solid: poorest prognosis, poorly distinguished.
➣Szanto grading:
• Grade I:Tubular/cribriform,
•Grade II: less than 30% solid.
•Grade III: more than 30% solid.
➣Perineural invasion, especially in its early phases, is a hallmark.
● Molecular Pathophysiology:
• t(6;9) → MYB-NFIB fusion oncogene is a genetic aberration.
• Apoptosis, adhesion, and the cell cycle are among the pathways impacted.
• Additional markers: FGFR1, EGFR, HER2, SOX4, c-KIT.
• Origin from intercalated ducts → differentiation of epithelium and myoepithelium.
● Histological analysis:
• Epithelial cells: positive for EMA and CEA.
• Myoepithelial cells: p63, calponin, S-100, and SMA.
• Cells that line the ducts: CD117 (c-KIT).
• Alcian blue positive (basement membrane material) and PAS pseudocysts.
• Prognostic indicators: p53 → recurrence; S-100, GFAP, and NCAM → perineural invasion.
Protocol
● Treatment
➣The gold standard is postoperative radiation combined with a wide surgical resection with distinct margins.
➣By site:
• Parotid → surgery (if feasible, maintain nerve) + radiotherapy.
• Submandibular → dissection of the supraomohyoid neck + RT + surgery.
• Radical excision with RT for minor salivary glands.
➣Dissection of the neck is not common (rare nodal spread).
➣Chemotherapy: Used in instances that are advanced or incurable; limited in scope.
● Pain Management
➣ Issue: Patients who suffer from chronic illnesses, underlying disorders, or surgery may find it difficult to function on a daily basis and maintain their general health.
➣ Management:
• Pharmacological: Giving prescription drugs, such as opioids, paracetamol, or NSAIDs, as directed. It is crucial to continuously check for adverse effects and efficacy.
• Non-pharmacological: Massage, physical therapy, deep breathing exercises, and teaching patients coping mechanisms.
➣ Nursing Interventions:
• Use approved pain scales to measure pain.
· Give prescriptions as directed.
• Provide relaxing techniques.
• Offer consolation and supportive care.
● Chronic Conditions (Diabetes, Hypertension)
➣Problem: To prevent complications and enhance quality of life, managing chronic diseases need continual education, observation, and assistance.
➣Management :
•includes routinely checking vital signs, such as blood pressure and glucose levels.
• Support for changing one's lifestyle, including diet and exercise regimens.
• Managing medications for the best possible illness control.
➣Nursing Interventions: • Inform patients about their disease, how to take their medications, and the warning signals of complications.
• Regularly check and record vital indicators.
Encourage patients to follow their diet and exercise regimens.
● Dyspnoea (Breathlessness)
➣ Issue: Patients with respiratory disorders who experience persistent dyspnoea have problems with their physical and mental stability.
➣Management:
• Pharmacological assistance (bronchodilators, oxygen treatment).
• Non-pharmacological methods (body posture, breathing techniques, fluid control).
➣Nursing interventions:
•include teaching energy-saving methods and breathing exercises.
• Promote hydration and dietary assistance.
• Monitor emotional well-being and offer self-management instruction.
● Nausea and Vomiting:
Issue: Nausea impacts patient comfort, medication absorption, and nutrition.
➣Management: Determine and address the root cause, administer antiemetics as directed, and promote frequent, little meals.
➣Nursing interventions:
• Track the occurrence and causes of bouts of nausea and vomiting.
· Give prescriptions as directed.
• Encourage hydration and comfort in the surroundings.
● Danger of Secondary Damage (such as in traumatic brain injury):
➣ Issue: Infections, pressure ulcers, and deteriorating neurological condition are among the secondary problems that critically sick patients, particularly those with brain damage, are susceptible to.
➣Management includes neuromonitoring, routine evaluation, and prompt action in response to conditional changes.
• To avoid problems, evidence-based practice and technology are used.
➣Nursing interventions include regular neurological examinations.
• Putting pressure ulcer prevention measures into practice.
• Education and assistance for families.
Notes
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