Primary pulmonary hyalinizing clear cell carcinoma with pseudopapillary structures and abundant cysts filled with mucus

Inflammatory and Nested Testicular Sex Cord Tumor

“…23,24 This is apparently the case for neoplasms driven by EWSR1::ATF1, a fusion that has been identified in multiple tumor types, including clear cell sarcoma of soft tissue, malignant gastrointestinal neuroectodermal tumor (also known as clear cell sarcoma-like tumor of the gastrointestinal tract), clear cell carcinoma of salivary gland, hyalinizing clear cell carcinoma of the thymus and lung, clear cell odontogenic carcinoma, myoepithelial tumors, intracranial myxoid mesenchymal tumor, primary pulmonary myxoid sarcoma, and subsets of mesothelioma, angiosarcoma, and angiomatoid fibrous histiocytoma. [25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41] These entities span a wide morphologic, biological, and clinical spectrum that comprises indolent entities with little malignant potential (angiomatoid fibrous histiocytoma), to low-grade malignancies (clear cell carcinoma of salivary gland), to aggressive neoplasms with poor outcomes (clear cell sarcoma of soft tissue, malignant gastrointestinal neuroectodermal tumor). IN-TSCTs seem to belong in the latter end of the spectrum, as they demonstrate a tendency for early metastatic spread to lymph nodes and visceral sites, with poor response to systemic therapy.…”

“…The phenotypic and biological features of tumors driven by gene fusions are likely determined by multiple factors, such as the cell of origin, the tumor microenvironment, and the presence of concurrent genomic and epigenomic alterations 23,24. This is apparently the case for neoplasms driven by EWSR1::ATF1 , a fusion that has been identified in multiple tumor types, including clear cell sarcoma of soft tissue, malignant gastrointestinal neuroectodermal tumor (also known as clear cell sarcoma-like tumor of the gastrointestinal tract), clear cell carcinoma of salivary gland, hyalinizing clear cell carcinoma of the thymus and lung, clear cell odontogenic carcinoma, myoepithelial tumors, intracranial myxoid mesenchymal tumor, primary pulmonary myxoid sarcoma, and subsets of mesothelioma, angiosarcoma, and angiomatoid fibrous histiocytoma 25–41. These entities span a wide morphologic, biological, and clinical spectrum that comprises indolent entities with little malignant potential (angiomatoid fibrous histiocytoma), to low-grade malignancies (clear cell carcinoma of salivary gland), to aggressive neoplasms with poor outcomes (clear cell sarcoma of soft tissue, malignant gastrointestinal neuroectodermal tumor).…”

Pulmonary salivary gland tumor–hyalinizing clear cell carcinoma: a literature review

“…Moreover, most pulmonary HCCC tumor cells have clear boundaries, however, under a microscope, it can be observed that pulmonary HCCC tumor cells invade adjacent alveolar parenchyma and lymph nodes around the bronchi or damages bronchial cartilage. In addition to the above situation, tumor cells were seen spreading along the bronchial wall below the respiratory epithelium in the case demonstrated by Feng et al [ 17 ]. Given the rarity of pulmonary HCCC, if these morphological details do not provide a definitive diagnosis, other techniques such as molecular studies or immunohistochemical markers can be applied [ 11 ].…”

“…Although pulmonary HCCC is similar to salivary gland HCCC and has a lower likelihood of metastasis, local recurrence may still occur [ 1 , 4 ]. In some cases of pulmonary HCCC, tumor cells infiltrating the lung parenchyma [ 14 ], cartilage [ 4 , 15 , 16 ], and nerves [ 5 , 16 , 17 ] have been confirmed, which may indicate an aggressive clinical course that can also lead to metastasis and recurrence [ 14 ]. For example, Wang et al [ 14 ] diagnosed a patient with pulmonary HCCC who underwent right upper lobe lobectomy.…”

Primary pulmonary hyalinizing clear cell carcinoma with fusions of both EWSR1::CREM and IRF2::NTRK3: report of a case with an aggressive behavior

“…reported a case initially misdiagnosed as moderately to poorly differentiated squamous cell carcinoma (SCC) with a biopsy specimen ( 4 ). Based on the bronchoscopic biopsy specimen, two cases were misdiagnosed as non-small cell lung cancer and squamous papillary neoplasm, respectively ( 5 , 12 ). The postoperative cases were also often diagnosed as other common tumors, such as MEC (1 case), SCC (2 cases), SCC/MEC (2 cases), and lung cancer (1 case) ( 2 – 4 , 6 , 10 ), which indicates a high misdiagnosis rate of pulmonary HCCC.…”