pleomorphic carcinoma

Pleomorphic carcinoma is considered to be a rare, very aggressive subtype that tends to present as a peripheral mass, frequently showing a mixture of spindle and giant cell features. Spindle cells and giant cells are often associated with other more common histological subtypes of lung carcinoma, supporting the concept that lung cancers are derived from a pluripotent stem cell that can exhibit differentiation into any potential combination of the various histological subtypes. Of all the potential combinations (spindle cell, giant cell, squamous cell, small cell and adenocarcinoma), spindle cell and giant cell subtypes showed the most frequent pattern of coexistence. Thus, tumors with bizarre or multinucleated cells in a spindle cell background are referred to as “pleomorphic,” a designation that was first applied by the AFIP.

Tumors fitting this description have also been reported to occur in the breast, prostate, gallbladder and pancreas. Pleomorphic carcinoma may present a diagnostic challenge for the surgical pathologist. The lesion is rare, and there is little opportunity to study many cases. The tumor must also be differentiated from a variety of other anaplastic pulmonary tumors, including sarcomas, carcinosarcomas, melanoma and metastatic tumors. Carcinosarcoma, in particular, is a similar biphasic tumor whose sarcomatous component demonstrates heterologous elements, such as malignant bone, cartilage or skeletal muscle. Immunohistochemical studies will help in the differentiation, the spindle cell component commonly staining for keratin, and vimentin, and variably for EMA and CEA. Apcalis Oral Jelly

The clinical behavior of pleomorphic lung cancer more closely parallels that of the large cell variant, including the propensity for gastrointestinal metastasis. It has, however, until recently, been classified as a variant of squamous cell carcinoma by the World Health Organization (WHO). In 1999, it was reclassified into a category of “Carcinoma with Pleomorphic, Sarcomatoid or Sarcomatous Elements” by the WHO/International Association for the Study of Lung Cancer (WHO/IASLC).

Pleomorphic carcinoma comprises only 0.3% of all lung malignancies. The male:female ratio varies from 3:1 to 6:1, with the median age of presentation at 59 years. As with other lung malignancies, smoking is a major risk factor. Sixty percent of these tumors present as a prepheral mass without endobronchial involvement, making it difficult, in some cases, to achieve a diagnosis with bronchoscopic examination or endobronchial biopsy. Approximately 50% of the cases present at stage III or IV, with chest wall invasion involving approximately 25% of the cases.

Our case is unique in its presentation, having symptomatic small-bowel metastasis prior to the discovery of the pulmonary lesion. Isolated small-bowel metastases from lung cancer have been considered a very rare event, with only 34 cases being reported prior to 1992. Additional cases have been reported as recently as 2003. In most of these cases, the patients were known to have metastatic prior to the occurrence of small-intestinal manifestations. To have the intestinal manifestations presenting prior to the detection of lung disease is still rarer.

Abdominal pain is the most common of the presenting symptoms (86%), followed by melena, and nausea and vomiting. Squamous cell carcinoma and large cell carcinoma are the most common subtypes of lung cancer that have been associated with intestinal metastasis, and the jejunum is the most com­mon site of metastases. Pleomorphic carcinoma, likewise, is noted to have a predilection for small-bowel metastases. In a recent article, Stenbygaard has also associated adenocarcinoma of lung with a propensity for small-intestine metastases.

Small-bowel metastases from lung cancer frequently present as intestinal perforation, with peritonitis and shock that is superimposed on the patient’s already-compromised performance status. These lesions result from bloodborne metastases that form mesenteric masses that infiltrate into the bowel wall, causing clinical symptoms. Lung cancer metastases have a tendency to undergo necrosis and consequent perforation. The earlier reported cases were associated with a dismal prognosis, with a mean survival time of 51 days. Many of the patients died in the perioperative period. It has been shown, however, in more recent cases that if palliative surgery is performed early enough to prevent perforation, the prognosis will be improved. A number of cases have shown better survival rates—one patient surviving nine months.
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Healthcare workers who are involved in the treatment of cancer patients should keep in mind that as life expectancy in general increases and as the life expectancy of lung cancer patients is prolonged by therapy, abdominal involvement by metastatic cancer can be expected to increase. Small-bowel metastases must be considered in any patient with both lung carcinoma and abdominal pain and should be expected in patients with both lung carcinoma and acute abdomen. Pathologists should be familiar with this unusual subtype of lung cancer and its behavior, as prompt diagnosis may facilitate earlier clinical intervention.