“…4,13 MASH1 is a basic helix-loop-helix transcription factor crucial for neuroendocrine differentiation. Recently, Ralston et al reported that 83.1% of pulmonary small cell carcinomas expressed MASH1 16 Only two additional cases of MCC that express both TTF-1 and CK20 have been reported. 14,17 Our Case 1 is the first documented case of MCC showing a CK20 − /TTF-1 + phenotype concurrent with Bowen's disease.…”
The concurrence of Merkel cell carcinoma (MCC) and squamous cell carcinoma (SCC) is well known, and MCC concurrent with Bowen's disease has also been documented. Herein, we describe two cases of MCC concurrent with Bowen's disease, and one case exhibited an unusual immunophenotype. An 86-year-old male (Patient 1) and an 87-year-old female (Patient 2) presented with nodules of the chest and cheek, respectively. Histopathologic study revealed Bowen's disease and a proliferation of small round cells in the dermis and/or subcutis. Immunohistochemically, the round cells expressed endocrine markers. 'Dot' immunopositivity for cytokeratin (CK) (AE1/AE3) was observed in both patients. However, dot-like CK20 positivity was present only in the second tumor, and thyroid transcription factor-1 (TTF-1) was only positive in the first. Both cases were negative for Merkel cell polyomavirus (MCPyV). MCC concurrent with SCC usually does not involve detectable MCPyV infection, which suggests that combined MCC may develop through different tumorigenetic pathways, such as chronic ultraviolet exposure, as compared to pure MCC. Additionally, concurrent tumors may exhibit an unusual immunophenotype, such as TTF-1(+) /CK20((-)) .
“…4,13 MASH1 is a basic helix-loop-helix transcription factor crucial for neuroendocrine differentiation. Recently, Ralston et al reported that 83.1% of pulmonary small cell carcinomas expressed MASH1 16 Only two additional cases of MCC that express both TTF-1 and CK20 have been reported. 14,17 Our Case 1 is the first documented case of MCC showing a CK20 − /TTF-1 + phenotype concurrent with Bowen's disease.…”
The concurrence of Merkel cell carcinoma (MCC) and squamous cell carcinoma (SCC) is well known, and MCC concurrent with Bowen's disease has also been documented. Herein, we describe two cases of MCC concurrent with Bowen's disease, and one case exhibited an unusual immunophenotype. An 86-year-old male (Patient 1) and an 87-year-old female (Patient 2) presented with nodules of the chest and cheek, respectively. Histopathologic study revealed Bowen's disease and a proliferation of small round cells in the dermis and/or subcutis. Immunohistochemically, the round cells expressed endocrine markers. 'Dot' immunopositivity for cytokeratin (CK) (AE1/AE3) was observed in both patients. However, dot-like CK20 positivity was present only in the second tumor, and thyroid transcription factor-1 (TTF-1) was only positive in the first. Both cases were negative for Merkel cell polyomavirus (MCPyV). MCC concurrent with SCC usually does not involve detectable MCPyV infection, which suggests that combined MCC may develop through different tumorigenetic pathways, such as chronic ultraviolet exposure, as compared to pure MCC. Additionally, concurrent tumors may exhibit an unusual immunophenotype, such as TTF-1(+) /CK20((-)) .
“…12 of our series). CK20 has become a standard marker for MCC and is positive in~95 % of cases showing a paranuclear dot-like pattern [23,24]; lack of CK20 expression has been described in 5 % of MCC [25][26][27][28]. The negativity of this marker in MCC is more likely due to the lack of detectable Merkel cell polyoma virus, as suggested in previous studies [28][29][30].…”
Merkel cell carcinoma (MCC) is an uncommon neuroendocrine small cell tumor derived from the transformation of the homonymous cells in the basal layer of the epidermis. MCC has a generally aggressive course, with a high tendency for local recurrence, lymph node involvement, and distant metastasis. Fine needle cytology (FNC) and immunocytochemistry were used as diagnostic procedures for 22 cases of MCC presented at our institute. All cases of MCC were successfully diagnosed on FNC. Among all of the monoclonal antisera used (CD56, CK20, CK MNF116, neuron-specific enolase (NSE), synaptophysin, and chromogranin), NSE and CD56 showed the highest frequency of positivity. The accuracy of the cytological diagnosis was 100% compared to the corresponding previous or subsequent pathological diagnoses. FNC and immunocytochemistry represent excellent and accurate diagnostic methods to distinguish MCC from other small-cell malignant entities.
“…Although CK20 negative primary MCCs or TTF-1 positive MCCs have been described, loss of CK20 and simultaneous acquisition of TTF-1 expression in metastatic MCC has not been reported. 1,2 …”
Merkel cell carcinoma is recognized by its morphologic features as well as by its classic immunophenotypic properties. Although Merkel cell carcinomas demonstrating non-classic immunoreactivities have been described, a case documenting a change in immunophenotype during the course of disease progression has not been previously reported. We report a case of Merkel cell carcinoma which initially demonstrated cytokeratin 20 positivity but lost expression in subsequent metastases. Likewise, thyroid transcription factor-1 was initially negative in the tumor but expression was present in metastatic lesions.
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