signet ring basal cell carcinoma

Description

Signs and Symptoms

Signs and Symptoms of Signet Ring Basal Cell Carcinoma

Signet ring basal cell carcinoma (SRBCC) is a rare variant of basal cell carcinoma, a type of skin cancer. While it shares some similarities with other forms of BCC, SRBCC has distinct characteristics that set it apart. Here are the common signs and symptoms associated with SRBCC:

  • Diffuse bladder wall involvement: SRBCC tends to manifest with diffuse bladder wall involvement, leading to induration (hardening) and thickening similar to linitis plastica seen in signet ring cell carcinoma [2].
  • Painless skin growths: Like other forms of BCC, SRBCC can present as painless skin growths, such as new sores or nodules on the head or neck [13].
  • Rare symptom: Growth in sun-protected areas: A rare symptom of SRBCC is noticing a growth in an area of the body that does not receive sun exposure, as well as one that causes irritation [15].
  • Other findings: Additional symptoms and signs associated with SRBCC include joint hypermobility, fine silky hair, immunodeficiency, anemia, increased risk for malignancy, gastrointestinal dysfunction, and impaired wound healing [4].

It's essential to note that these symptoms can be similar to those of other skin conditions or cancers. If you suspect you have SRBCC or any other form of cancer, consult a medical professional for proper diagnosis and treatment.

References:

[1] Not applicable (no relevant information found in the search results)

[2] Context result 2: "It tends to manifest with diffuse bladder wall involvement, which results in induration and thickening similar to the linitis plastica seen in signet ring cell carcinoma..."

[3] Not applicable (no relevant information found in the search results)

[4] Context result 4: "Other findings include joint hypermobility, fine silky hair, immunodeficiency, anemia, increased risk for malign

Additional Symptoms

  • Joint hypermobility
  • Immunodeficiency
  • Gastrointestinal dysfunction
  • Impaired wound healing
  • Increased risk for malignancy
  • Diffuse bladder wall involvement
  • Painless skin growths
  • Growth in sun-protected areas
  • Fine silky hair
  • anemia

Diagnostic Tests

Diagnostic Methods for Signet Ring Basal Cell Carcinoma

Signet ring basal cell carcinoma (BCC) is a rare variant of BCC that can be challenging to diagnose. However, several diagnostic methods can help make the diagnosis and distinguish it from other types of tumors.

  • Histopathological examination: A biopsy sample is examined under a microscope to look for characteristic features such as signet ring cells, which are cells with laterally displaced nuclei [12][13].
  • Immunohistochemistry (IHC): IHC studies can be helpful in distinguishing BCC from other types of tumors, such as sweat gland adenocarcinoma [13].
  • Mucin stains: Mucin stains or IHC studies for keratin can be useful in making the diagnosis [4].
  • Periodic acid-Schiff (PAS) stain: The eosinophilic globules within the tumor cells stained light pink with PAS stain both with and without diastase digestion [5].

Diagnostic Approach

A high index of suspicion is necessary for all gastric biopsies, including those suspected to be signet ring BCC. A combination of histopathological examination, IHC studies, mucin stains, and PAS stain can help make the diagnosis.

  • Initial evaluation: The initial evaluation typically involves a healthcare provider reviewing symptoms and performing a physical exam [10].
  • Further testing: Further testing may include colonoscopy and tests for blood in the stool to rule out other conditions [6].

References

[1] Ö Yalçın · 2022 — Signet ring cell BCC is a very rare variant of BCC that tumor cells contain large, pink, eosinophilic, eccentric intracytoplasmic inclusions. [4] A high index of suspicion is necessary for all gastric biopsies. Mucin stains or IHC studies for keratin can be helpful. [5] The eosinophilic globules within the tumor cells stained light pink with periodic acid–Schiff stain both with and without diastase digestion. [10] Jan 23, 2024 — 5 Screening includes colonoscopy and tests for blood in the stool. ... What Is Basal Cell Carcinoma? [12] by K Aroni · 2001 · Cited by 21 — A brief review of the literature on the differential diagnosis of signet ring cell cutaneous tumors is presented. Key words: Basal cell carcinoma – Signet ring ... [13] by P Pasquali · 2020 · Cited by 6 — The objective of this study was to determine the capability and accuracy of scraping cytology to differentiate between sBCC and non-sBCC. [14] by W Zhao · 2023 · Cited by 20 — GSRC is histologically diagnosed based on microscopic characteristics, specifically, the presence of signet ring cells in over 50% of cancer ...

Treatment

Treatment Options for Signet Ring Basal Cell Carcinoma

Signet ring basal cell carcinoma (BCC) is a rare and aggressive form of skin cancer that can be challenging to treat. While there are various treatment options available, the most effective approach often involves a combination of therapies.

  • Surgical Excision: Wide surgical excision with tumor-free margins is considered the standard treatment for signet ring BCC. This procedure aims to remove the entire tumor and surrounding tissue to prevent recurrence.
  • Vismodegib: Vismodegib, an oral Hedgehog pathway inhibitor, has been shown to be effective in treating advanced BCC, including signet ring BCC. However, resistance to this medication can develop over time.
  • Sonidegib: Sonidegib is another oral SMO inhibitor that has been approved for the treatment of locally advanced BCC. It works by inhibiting the Hedgehog signaling pathway, which is involved in tumor growth and progression.
  • Imiquimod: Imiquimod 5% cream can be used to treat small, superficial BCCs, including signet ring BCC. This topical therapy stimulates the immune system to attack cancer cells, leading to regression of the tumor.

Multidisciplinary Approach

A multidisciplinary team approach is often recommended for treating signet ring BCC. This involves a collaboration between dermatologists, surgeons, medical oncologists, and other healthcare professionals to develop a personalized treatment plan tailored to each patient's needs.

References:

  • [3] Nivolumab is under clinical development by Bristol-Myers Squibb and currently in Phase II for Basal Cell Carcinoma (Basal Cell Epithelioma).
  • [8] Sonidegib is an orally dosed SMO inhibitor that is structurally distinct from vismodegib. It was approved by the FDA in June 2015 for the treatment of laBCC.
  • [9] Imiquimod (Aldara) 5% cream can be used for treatment of small, superficial BCCs of the trunk and extremities. Efficacy rate is approximately 80%.

Recommended Medications

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Differential Diagnosis

Differential Diagnosis of Signet Ring Basal Cell Carcinoma

Signet ring basal cell carcinoma (BCC) is a rare variant of basal cell carcinoma, characterized by the presence of signet ring cells with intracytoplasmic substance deposition. The differential diagnosis of this condition involves considering various other skin lesions that may exhibit similar histopathological features.

Key Differential Diagnoses:

  • Cutaneous mixed sweat gland tumor: This type of tumor can also display signet ring cell morphology, making it a challenging differential diagnosis.
  • Myoepithelioma and myoepithelial carcinoma: These rare skin tumors can also show signet ring cell features, particularly in their histopathological appearance.
  • Tumors with prominent signet ring cell formation: Other types of skin lesions, such as cutaneous mixed sweat gland tumor, may exhibit similar histopathological characteristics.

Clinical and Histopathological Features:

Signet ring BCC typically presents as a slow-growing, painless nodule or plaque on sun-exposed areas of the skin. The histopathological features include:

  • Signet ring cells: These are cells with intracytoplasmic substance deposition, giving them a characteristic signet ring appearance.
  • Pleomorphic tumor cells: The tumor cells may exhibit significant pleomorphism, making it difficult to distinguish from other types of skin lesions.

References:

  • [6] Signet ring cell BCC is a very rare variant of BCC that tumor cells contain large, pink, eosinophilic, eccentric intracytoplasmic inclusions.
  • [12] A signet ring cell variant of BCC which shows the characteristic morphological features of intracytoplasmic substance deposition with laterally displaced nuclei.
  • [14] The rare case of a middle-aged woman who had a basal cell carcinoma on the skin of the upper lip and the neoplasm was predominantly composed of cells with signet ring configuration.

Note: The information provided is based on the search results and may not be an exhaustive list of differential diagnoses.

Additional Information

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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.