Preferred Label : Small cell cancer of the lung;
CISMeF acronym : SCCL; SCLC1;
Type : Phenotype or locus, molecular basis unknown;
Alternative titles and symbols : SCCL; SCLC1; Sclc;
Description : Small cell cancer of the lung accounts for about a fourth of the 110,000 new cases
of lung cancer that occur annually in the United States. It is clinically distinctive:
usually metastases are already present at the time of discovery so that surgery is
not used. In contrast to adeno- and squamous carcinoma, SCCL is sensitive to chemotherapy
and radiotherapy. Whang-Peng et al. (1982) found a specific, acquired chromosomal
abnormality (deletion 3p) in at least one chromosome 3 in all metaphases in all 12
cell lines cultured from human SCCL tissue in 2-day tumor culture specimens from 3
patients. The shortest region of overlap showed the deletion to involve 3p23-p14.
No other type of lung cancer showed this deletion, nor did lymphoblastoid lines cultured
from SCCL patients whose tumors had the 3p deletion. SCCL is 'caused' by cigarette
smoking as are other types of lung cancer. Thus, like chronic myeloid leukemia, this
is an example of an exogenously induced malignancy with a specific chromosomal change.
Cytogenetic effects of cigarette smoke are relevant in this connection (Madle et al.,
1981). Several biochemical markers were found to be associated with small cell cancer
of the lung (Gazdar et al., 1981; Tapia et al., 1981). Perhaps genes in the 3p14-23
region have something to do with these markers as well as with the genesis of SCCL.
(The cell of origin of SCCL is thought to be the Kulchitsky cell, an argentaffine
cell situated in the bronchial epithelium, although this is not proved.) Erisman et
al. (1982) showed that SCCL contains bombesin, a tetradecapeptide from anuran skin.
It had been identified in human fetal and neonatal lung but not in adult lung. Some
symptoms of SCCL may be attributable to bombesin. The syndrome of inappropriate secretion
of antidiuretic hormone and Cushing syndrome, occurring with SCCL, are due to ectopic
production of antidiuretic hormone and ACTH, respectively. The relation between ectopic
hormone production and the aberration involving chromosome 3 is unknown. Baylin et
al. (1982) found 12 distinguishing surface proteins on SCCL that were not shared by
any of the 3 other carcinogen-induced forms of lung cancer (squamous, adeno-, and
large cell undifferentiated carcinoma) or by human lymphoblastoid cells and fibroblasts.
The neuroendocrine nature of SCCL was supported by the fact that 6 of the 12 were
shared by human neuroblastoma cells. On human SCCL cells and tumors, Ruff and Pert
(1984) demonstrated 4 surface antigens previously recognized only in macrophages.
They suggested that cancerous cells may arise from macrophage precursors in bone marrow,
and these precursors migrate to lung to participate in the repair of tissue damage
produced by continuous heavy smoking. About 5% of SCCL patients have no apparent pulmonary
involvement and the early, rapid and widespread dissemination of tumor to extrathoracic
sites requires explanation. Naylor et al. (1984) used an anonymous, polymorphic DNA
probe, D3S3, to confirm the presence of deletion of 3p in SCCL. This probe had been
assigned to 3p21-cen. Studying 7 SCCL tumors and normal tissue from the same persons,
they found that 6 of the 'normal' DNA samples were heterozygous for the D3S3 MspI
polymorphism, whereas in all cases the tumor tissues were homozygous. De Leij et al.
(1985) isolated 3 new, well-growing cell lines from SCCL. Deletions in 3p, with 3p23-p21
as the smallest region of overlap, were found. Mooibroek et al. (1987) used a recombinant
DNA fragment detecting a RFLP presumably at 3p21 to probe DNA isolated from leukocytes
of 12 patients with small cell lung cancer. Four of these patients were heterozygous.
Analysis of tumor material from the 4 patients showed homozygosity for either one
or the other restriction fragment in every case. Gerber and Scoggin (1987) and Naylor
et al. (1987) demonstrated loss of constitutional heterozygosity in;
Inheritance : Autosomal dominant;
Prefixed ID : %182280;
Origin ID : 182280;
UMLS CUI : C0149925;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- DO Cross reference
- Genes related to phenotype
- HPO term(s)
- ORDO concept(s)
- Semantic type(s)
- UMLS correspondences (same concept)
