Acinic cell carcinoma of the lung | |
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Other names | Fechner tumor, AcCC of the lung |
Very high magnification micrograph of an acinic cell carcinoma, abbreviated AcCC. H&E stain. | |
Specialty | Oncology, Pulmonology |
Acinic cell carcinoma of the lung is a very uncommon tumor that typically appears close to the right bronchus. [1] As of 2022, only 29 cases have been documented in the English literature [1] since Fechner et al. first described this entity in 1972. [2] Histologically similar to the major and minor salivary glands, pluripotent cells of the submucosal serous and mucous glands of the tracheobronchial tree are thought to be the source of acinic cell carcinoma of the lung. The histologic characteristics of acinic cell carcinoma of the lung are nearly identical to those of the salivary glands. [3]
Hemostasis and cough were the most common clinical symptoms. Many times, patients have no symptoms. Tumors were typically small, seldom growing larger than 4 cm. The right bronchus was most likely to be affected by the tumor, then the left bronchus and trachea. [4]
Prior to making a diagnosis of primary acinic cell carcinoma of the lung, a comprehensive clinical history, physical examination, and imaging studies are necessary due to the indolent nature of primary acinic cell carcinoma of the salivary gland and its potential for metastasis after extended latency. [5]
Primary acinic cell carcinoma of the lung is particularly difficult to diagnose histologically because the primary basis for the diagnosis is morphological features. [1] Fechner et al. initially reported on two cell populations in primary acinic cell carcinoma of the lung: a majority of light cells, akin to acinic cell tumor of the parotid gland, and dark cells, resembling normal serous cells of the bronchial submucosal glands. [2]
When compared to the traditional "blue dot" appearance of acinic cell carcinomas originating from the parotid gland, [6] the majority of primary pulmonary acinic cell carcinomas exhibit a mixture of clear and eosinophilic granular cytoplasm. This discrepancy can be attributed to the pulmonary submucosal gland's serous cells secreting a low concentration of peptides, proteins, and small organic molecules acting as antimicrobials, as well as the fact that they do not produce digestive zymogen. [7] As a result, PAS-D stain weak positivity or negativity was noted. [8] [9] When assessing pulmonary ACC, immunohistochemical stains are not very useful because they show an immunoprofile that is typical of serous epithelial cells. [9] EMA and cytokeratins typically cause immunoreactivity in these tumors. S100, lysozyme, and chromogranin are all negative. [9] [8] Amylase and α-1-antichymotrypsin have unreliable immunoreactivity. [4]
The parotid gland is a major salivary gland in many animals. In humans, the two parotid glands are present on either side of the mouth and in front of both ears. They are the largest of the salivary glands. Each parotid is wrapped around the mandibular ramus, and secretes serous saliva through the parotid duct into the mouth, to facilitate mastication and swallowing and to begin the digestion of starches. There are also two other types of salivary glands; they are submandibular and sublingual glands. Sometimes accessory parotid glands are found close to the main parotid glands.
Surface epithelial-stromal tumors are a class of ovarian neoplasms that may be benign or malignant. Neoplasms in this group are thought to be derived from the ovarian surface epithelium or from ectopic endometrial or Fallopian tube (tubal) tissue. Tumors of this type are also called ovarian adenocarcinoma. This group of tumors accounts for 90% to 95% of all cases of ovarian cancer; however is mainly only found in postmenopausal women with the exception of the United States where 7% of cases occur in women under the age of 40. Serum CA-125 is often elevated but is only 50% accurate so it is not a useful tumor marker to assess the progress of treatment. 75% of women with epithelial ovarian cancer are found within the advanced-stages; however younger patients are more likely to have better prognoses than older patients.
Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, Bartholin gland, trachea, and the paranasal sinuses.
Canalicular adenoma is a benign, epithelial salivary gland neoplasm arranged in interconnecting cords of columnar cells. This is a very rare benign neoplasm, that makes up about 1% of all salivary gland tumors, or about 4% of all benign salivary gland tumors.
Mucoepidermoid carcinoma (MEC) is the most common type of minor salivary gland malignancy in adults. Mucoepidermoid carcinoma can also be found in other organs, such as bronchi, lacrimal sac, and thyroid gland.
Acinic cell carcinoma is a malignant tumor representing 2% of all salivary tumors. 90% of the time found in the parotid gland, 10% intraorally on buccal mucosa or palate. The disease presents as a slow growing mass, associated with pain or tenderness in 50% of the cases. Often appears pseudoencapsulated.
Carcinosarcomas are malignant tumors that consist of a mixture of carcinoma and sarcoma. Carcinosarcomas are rare tumors, and can arise in diverse organs, such as the skin, salivary glands, lungs, the esophagus, pancreas, colon, uterus and ovaries.
Salivary gland tumours, also known as mucous gland adenomas or neoplasms, are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular, and sublingual glands. The minor salivary glands consist of 800 to 1000 small mucus-secreting glands located throughout the lining of the oral cavity. Patients with these types of tumours may be asymptomatic.
Large cell lung carcinoma with rhabdoid phenotype (LCLC-RP) is a rare histological form of lung cancer, currently classified as a variant of large cell lung carcinoma (LCLC). In order for a LCLC to be subclassified as the rhabdoid phenotype variant, at least 10% of the malignant tumor cells must contain distinctive structures composed of tangled intermediate filaments that displace the cell nucleus outward toward the cell membrane. The whorled eosinophilic inclusions in LCLC-RP cells give it a microscopic resemblance to malignant cells found in rhabdomyosarcoma (RMS), a rare neoplasm arising from transformed skeletal muscle. Despite their microscopic similarities, LCLC-RP is not associated with rhabdomyosarcoma.
Epithelial-myoepithelial carcinoma of the lung is a very rare histologic form of malignant epithelial neoplasm ("carcinoma") arising from lung tissue.
Sarcomatoid carcinoma of the lung is a term that encompasses five distinct histological subtypes of lung cancer, including (1) pleomorphic carcinoma, (2) spindle cell carcinoma, (3) giant cell carcinoma, (4) carcinosarcoma, or (5) pulmonary blastoma.
Giant-cell carcinoma of the lung (GCCL) is a rare histological form of large-cell lung carcinoma, a subtype of undifferentiated lung cancer, traditionally classified within the non-small-cell lung carcinomas (NSCLC).
Salivary gland–like carcinomas of the lung generally refers a class of rare cancers that arise from the uncontrolled cell division (mitosis) of mutated cancer stem cells in lung tissue. They take their name partly from the appearance of their abnormal cells, whose structure and features closely resemble those of cancers that form in the major salivary glands of the head and neck. Carcinoma is a term for malignant neoplasms derived from cells of epithelial lineage, and/or that exhibit cytological or tissue architectural features characteristically found in epithelial cells.
Basaloid squamous cell carcinoma (Bas-SqCC) is an uncommon histological variant of lung cancer composed of cells exhibiting cytological and tissue architectural features of both squamous cell lung carcinoma and basal cell carcinoma.
Myoepithelioma of the head and neck, also myoepithelioma, is a salivary gland tumour of the head and neck that is usually benign. When malignant, which is exceedingly rare, they are known as malignant myoepithelioma or Myoepithelial carcinoma, and they account for 1% of the salivary tumors with poor prognosis.
Epithelial-myoepithelial carcinoma (EMCa) is a rare malignant tumour that typically arises in a salivary gland and consists of both an epithelial and myoepithelial component. They are predominantly found in the parotid gland and represent approximately 1% of salivary gland tumours.
A sialoblastoma is a low-grade salivary gland neoplasm that recapitulates primitive salivary gland anlage. It has previously been referred to as congenital basal cell adenoma, embryoma, or basaloid adenocarcinoma. It is an extremely rare tumor, with less than 100 cases reported worldwide.
Ceruminous adenocarcinoma is a malignant neoplasm derived from ceruminous glands of the external auditory canal. This tumor is rare, with several names used in the past. Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), and ceruminous mucoepidermoid carcinoma.
Sclerosing polycystic adenosis is a rare salivary gland tumor first described in 1996 by Dr. Brion Smith. The major salivary glands, specifically the parotid gland and the submandibular gland, are affected most commonly. Patients usually come to clinical attention with a mass or swelling in their salivary glands in the 5th decade of life, with females affected much more commonly than males. Nearly all of the cases reported so far have a benign behavior, although there is a single case that has had an associated malignant transformation.
Mammary analogue secretory carcinoma (MASC), also termed MASCSG, is a salivary gland neoplasm. It is a secretory carcinoma which shares the microscopic pathologic features with other types of secretory carcinomas including mammary secretory carcinoma, secretory carcinoma of the skin, and salivary gland–type carcinoma of the thyroid. MASCSG was first described by Skálová et al. in 2010. The authors of this report found a chromosome translocation in certain salivary gland tumors, i.e. a (12;15)(p13;q25) fusion gene mutation. The other secretory carcinoma types carry this fusion gene.