Acinar cell carcinoma of the pancreas ACC/PACC)

The Acinar cell carcinoma of the pancreas (engl. pancreatic acinar cell carcinoma, PACC) is a rarer malignant Tumor of the exocrine pancreas. It arises from the acinar cells, which produce digestive enzymes, and accounts for only a small proportion of pancreatic tumors.

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Brief overview

• Rarity: significantly rarer than the ductal Adenocarcinoma; PACC is usually described in reviews as <2% of pancreatic carcinomas.
• Age/gender: More common in men; typical diagnoses around middle to older adulthood, but in principle a broad age distribution is possible.
• Biology: differs clinically and molecularly from ductal Pancreatic carcinoma, which may be relevant for treatment decisions.

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What makes acinar cell carcinoma special?

Compared to the common ductal pancreatic carcinoma, PACC:

• Grow differently and spread differently,
• larger tumors at the time of diagnosis,
• and partly other molecular changes which opens the door for Targeted therapies can be opened in selected cases (depending on the findings).

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Symptoms

Many symptoms are non-specific and resemble other pancreatic diseases. Frequently mentioned are

• Upper abdominal pain, feeling of pressure
• Weight loss, loss of appetite
• Nausea/feeling of fullness
• Tiredness/performance slump

In some cases, this can lead to Lipase increase (enzyme production), sometimes with particular side effects (e.g. fatty tissue reactions).

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Diagnostics

Typical steps (depending on the situation):

1. Imaging: CT/MRI (if necessary PET-CT), assessment of size, localization, Metastases.
2. Endosonography (EUS) + Biopsy: histologic backup.
3. Laboratory: Liver values, bilirubin, inflammation values; lipase/amylase if necessary.
4. Molecular diagnostics: can be particularly valuable in PACC when systemic therapies are planned.

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Therapy (conventional medical standard - overview)

Treatment depends on the stage, operability and metastasis:

• Operation (if possible) is usually the most important option for localized disease.
• System therapy (chemotherapy) for advanced/metastatic stages; regimens are selected individually.
• Personalized/target approaches may be considered depending on the molecular profile (case-by-case decision).
• Supportive therapy: Pain therapy, nutritional medicine, enzyme substitution (exocrine pancreatic insufficiency), psycho-oncological support.

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Forecast

The prognosis depends heavily on:

• Diagnosis stage (local vs. metastatic),
• complete resection (R0),
• Tumor biology/molecular profile
  from. Overall, PACC is rare, so much of the data comes from reviews and case series.

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Frequency therapy section: Frequency lists (EDTFL / CAFL)

1) CAFL (Consolidated Annotated Frequency List)

There is an entry in the publicly available CAFL PDF:

• Cancer_pancreatic: „use Cancer_adenocarcinoma“
• Cancer_adenocarcinoma (Hz):
  47 | 832 | 2084 | 2127 | 2160 | 2182 | 2219 | 2452 | 2876

Note on classification: The Acinar cell carcinoma is histologically not identical to a ductal adenocarcinoma. In CAFL however, „pancreatic carcinoma“ is pragmatically mapped via the adenocarcinoma entry.

2) EDTFL (ETDFL) - availability/research

EDTFL/ETDFL is offered as a digital, searchable frequency list; concrete disease-specific frequency sets are often not available, depending on the version. only within the respective PDF/database reliably visible.

I could not find a clear, citable EDTFL set for „acinar cell carcinoma (pancreas)“ in freely accessible sources. In practice, the above-mentioned CAFL-Pancreas/Cancer_adenocarcinoma frequencies until a specific EDTFL entry can be looked up in its own EDTFL PDF/database.

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Important note (disclaimer)

The frequency lists mentioned here originate from complementary medicine collections (e.g. CAFL/EDTFL) and are not part of the evidence-based standard therapy of pancreatic carcinomas. Frequency therapy is not recognized by conventional medicine and can do not replace treatment by doctors or alternative practitioners. Please only use this information as a guide and always seek medical advice for diagnosis and treatment.

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Author: NLS Information medicine Ltd, Herbert Eder