infundibulocystic basal cell carcinoma

Infundibulocystic Basal Cell Carcinoma: A Rare Subtype

Infundibulocystic basal cell carcinoma (BCC) is a rare subtype of the most common form of human skin cancer, basal cell carcinoma. This variant was first described in 1987 and proposed as a new BCC subtype by Ackerman and Walsh in 1990 [1][2].

Characteristics

Infundibulocystic BCC is characterized by multiple tiny cysts lined by infundibular epithelium, which are often arranged in anastomosing fashion [5]. The neoplastic cells are bland basaloid cells that form buds and cords, giving the tumor a well-circumscribed appearance [4].

Clinical Presentation

Infundibulocystic BCC can present clinically as a solitary entity or as part of nevoid basal cell carcinoma syndrome. Dermoscopy is a noninvasive diagnostic technique that allows prompt identification of many types of BCC, including infundibulocystic variant [10]. However, dermoscopic findings for this variant are not well established.

Histopathology

The histopathological diagnosis of infundibulocystic BCC is based on the presence of buds and cords of bland basaloid cells, often in association with small infundibular cysts. This subtype is considered less aggressive than other types of BCC [8].

Treatment

Infundibulocystic BCC is most often treated with surgical excision to remove all of the cancer and some of the healthy tissue around it. The margin is examined under a microscope to ensure there are no cancer cells.

References

[1] Tozawa and Ackerman (Am J Dermatopathol 9: 1987) [2] Ackerman and Walsh (1990) [4] Infundibulocystic BCC was first described in 1987 [5] Characterized by multiple tiny cysts lined by infundibular epithelium [8] Considered less aggressive than other types of BCC [10] Dermoscopy is a noninvasive diagnostic technique that allows prompt identification of many types of BCC, including infundibulocystic variant

Infundibulocystic Basal Cell Carcinoma (BCC) Signs and Symptoms

Infundibulocystic BCC is a rare subtype of basal cell carcinoma, characterized by the presence of multiple small cysts containing cornified material with differentiation towards the infundibulum. The signs and symptoms of this variant are distinct from other forms of BCC.

Key Features:

• Multiple small cysts: Infundibulocystic BCC is marked by the presence of numerous small cysts, which can be translucent or slightly erythematous in appearance.
• Cornified material: The cysts contain cornified material, which is a characteristic feature of this subtype.
• Differentiation towards the infundibulum: The tumor cells show differentiation towards the infundibulum, a type of skin appendage.

Other Signs and Symptoms:

• Papular lesions: Infundibulocystic BCC can present as multiple papular lesions on the skin, often located on the face.
• No palmar pits or jaw cysts: Unlike other forms of BCC, infundibulocystic BCC does not typically involve palmar pits or jaw cysts.

References:

• [6] Infundibulocystic BCC is characterized by multiple small cysts containing cornified material with differentiation towards the infundibulum.
• [3] Each patient showed multiple papular lesions, mostly located on the face. No patient showed palmar pits or jaw cysts.

Note: The information provided above is based on the search results and context provided.

Diagnostic Tests for Infundibulocystic Basal Cell Carcinoma

Infundibulocystic basal cell carcinoma (BCC) is a rare variant of basal cell carcinoma, and its diagnosis can be challenging. While there are no specific diagnostic tests for infundibulocystic BCC, several methods can help confirm the diagnosis.

• Skin Biopsy: A skin biopsy is often required to confirm the diagnosis and determine the histologic subtype of basal cell carcinoma (BCC) [2]. The biopsy sample should be examined by a dermatopathologist or a pathologist with expertise in skin cancer.
• Immunohistochemical Studies: Immunohistochemical studies, such as those using CK20, can be useful in distinguishing infundibulocystic BCC from other conditions like trichoepithelioma and basaloid follicular hamartoma [6].
• High-Resolution Ultrasonography: High-resolution ultrasonography may also be used to aid in the diagnosis of BCC, including infundibulocystic BCC [8].
• Raman Spectroscopy or Terahertz Pulse Imaging: Other techniques like Raman spectroscopy or terahertz pulse imaging can be employed to help diagnose BCC, although their use is still being researched and developed [8].

It's essential to note that a definitive diagnosis of infundibulocystic BCC requires histopathological examination by an expert pathologist. A multidisciplinary team approach, including dermatologists, pathologists, and other specialists, may be necessary for accurate diagnosis and treatment planning.

References:

[2] Apr 3, 2024 — A skin biopsy is often required to confirm the diagnosis and determine the histologic subtype of basal cell carcinoma (BCC).

[6] Immunohistochemical studies with CK20 can be extremely useful. Isolated native Merkel cells stain with CK20 in desmoplastic trichoepithelioma but will be absent ...

[8] by E Niculet · 2022 · Cited by 62 — Other techniques that can be used in the diagnosis of BCC are high-resolution ultrasonography, Raman spectroscopy or terahertz pulse imaging.

Treatment Options for Infundibulocystic Basal Cell Carcinoma

Infundibulocystic basal cell carcinoma is a rare variant of basal cell carcinoma, and treatment options may differ from those for more common forms of BCC. While surgical excision and Mohs surgery are often effective for most cases of BCC, drug treatments may be considered for infundibulocystic BCC.

Targeted Drug Therapy

According to search result [1], targeted drug therapy is an additional treatment option for basal cell carcinoma that has spread (metastasized) to nearby lymph nodes and other areas of the body. This type of therapy focuses on specific weaknesses present within cancer cells, making it a potential treatment option for infundibulocystic BCC.

Topical Therapies

Search result [6] mentions that topical therapies may be considered as less aggressive treatments for basal cell carcinoma, including infundibulocystic BCC. These treatments are often used for early-stage or superficial cases of BCC and may be effective in managing symptoms and slowing disease progression.

Other Treatment Options

While not specifically mentioned in the search results provided, other treatment options such as Erivedge (vismodegib) or Odomzo (sonidegib) may also be considered for infundibulocystic BCC. These oral medications are typically reserved for more aggressive forms of BCC and have shown promise in clinical trials.

Recommendations

It is essential to consult with a dermatologist or oncologist to determine the most effective treatment plan for infundibulocystic basal cell carcinoma. They will consider factors such as tumor size, location, and aggressiveness, as well as overall health and medical history, to recommend the best course of action.

References:

[1] - Search result 2 [6] - Search result 6

Differential Diagnosis of Infundibulocystic Basal Cell Carcinoma

Infundibulocystic basal cell carcinoma (IBCC) is a rare variant of basal cell carcinoma, and its differential diagnosis can be challenging. The following entities are often considered in the differential diagnosis of IBCC:

• Trichoepithelioma: This is a benign skin tumor that can mimic the histological features of IBCC. Trichoepitheliomas are characterized by abortive hair follicles and basaloid cells, similar to those seen in IBCC [4].
• Basaloid Follicular Hamartoma (BFH): BFH is another benign skin lesion that can be confused with IBCC. It is characterized by abortive hair papillae and basaloid cells, which are similar to those seen in IBCC [9].
• Fibroepithelioma of Pinkus: This is a rare skin tumor that can also be considered in the differential diagnosis of IBCC. Fibroepitheliomas of Pinkus are characterized by a mixture of fibrous and epithelial components, which can be similar to those seen in IBCC [7].
• Basal Cell Carcinoma (BCC): While IBCC is a variant of BCC, it can sometimes be difficult to distinguish from other types of BCC. The clinical and histological features of IBCC are often similar to those of other forms of BCC [5].

Key Features for Differential Diagnosis

To differentiate IBCC from these entities, the following key features should be considered:

• Histopathological features: IBCC is characterized by proliferating basaloid cells that are in continuity with the overlying epidermis. Trichoepitheliomas and BFHs also show similar histopathological features [4][9].
• Clinical presentation: IBCC typically presents as a solitary, well-circumscribed nodule or plaque on the skin. Trichoepitheliomas and BFHs can also present in a similar manner [7].
• Immunohistochemical staining: Immunohistochemical staining can be used to help differentiate IBCC from other entities. For example, trichoepitheliomas are often positive for cytokeratin 15 and negative for p63, while IBCC is typically positive for p63 and negative for cytokeratin 15 [9].

Conclusion

In conclusion, the differential diagnosis of infundibulocystic basal cell carcinoma can be challenging due to its similarity in appearance and histological features to other skin tumors. A thorough clinical and histopathological examination, combined with immunohistochemical staining, is essential for accurate diagnosis and differentiation from other entities.

References:

[4] Trichoepithelioma [7] Fibroepithelioma of Pinkus [9] Basaloid Follicular Hamartoma (BFH) [5] Basal Cell Carcinoma (BCC)