Journal of Surgical Oncology 19:216-218 (1982)

Carcinosarcoma of the Lung ~

DEBA P. SARMA, MD, AND SELDON J. DESHOTELS, JR, MD, MS

Department of Pathology, Louisiana State University Medical School and Veterans Administration Medical Center, New Orleans

A case of primary carcinosarcoma of lung is described and the literature is reviewed. The histogenesis of this rare tumor is discussed.

KEY WORDS: mixed malignant tumor of lung, pulmonary carcinosarcoma, lung cancer

INTRODUCTION Carcinosarcoma of the lung is a rare tumor presenting as

an endobronchial mass with variable histologic features, most commonly consisting of an epidermoid carcinoma with a sarcomatoid component [ 11. Clinically, there seems to be a relation between biologic behavior and location of the tumor. Centrally located lesions typically are pedunculated and present with obstructive symptoms. The peripherally located tumors tend to have a somewhat poorer prognosis [ 2 4 ] . Classification of these tumors has been controversial and histogenesis uncertain [5, 61.

The purpose of this report is to present a case of primary lung carcinosarcoma with widespread metastases and to discuss the histogenesis of such tumors.

CASE REPORT A 52-year-old while male was admitted with recent onset

of numbness in the right leg that progressed to his right arm accompanied by right-sided weakness. Pertinent physical examination revealed a thin, elderly male in moderate res- piratory distress, hepatomegaly , and neurologic findings of right hemiparesis, and decreased muscle tone. No patho- logic reflexes were elicited. Cranial nerves were intact ex- cept for slight ptosis of the left eye. Laboratory data revealed an anemia (hemoglobin 10.4 gm/dl), and sputum analysis with culture showed an enterococcus species and Klebsiella pneumonia. Chest roentgenograms revealed diffuse emphy- sema and a 4.0 cm mass in the left upper lung field. Brain scan indicated a left cerebral infarct, and electroencephal- ographic studies showed slow activity over the left fron- totemporal area.

The patient was admitted with a diagnosis of cerebro- vascular accident and probable bronchogenic carcinoma. A metastatic work-up included a biopsy of a nodule in the left mandibular area showing metastatic undifferentiated car- cinoma. The patient developed focal seizures and slowly deteriorated until he expired nineteen days after admission.

0022-4790/82/1904-0216$01.50 0 1982 Alan R. Liss, Inc.

At autopsy, both lungs showed dense pleural adhesions and numerous bilateral upper lobe emphysematous blebs. In the subapical area of the left upper lobe was a 3.0 cm tumor that was firm and grayish-red. The tumor commu- nicated with the bronchus in the area. The liver was enlarged (2,375 gm) and showed numerous soft, dark-red hemor- rhagic tumor nodules. One mass at the lower right lobe border had ruptured into the peritoneal cavity. A 10-cm mass was found near the ligament of Trietz in the mesentery infiltrating the small intestine. The left kidney, left adrenal gland and brain contained multiple metastatic tumor nod- ules. Microscopic examination of the lung tumor revealed a polypoid intrabronchial growth pattern with areas of ep- idermoid carcinoma with focal adenoid cystic differentiation combined with a sarcomatous differentiation resembling osteogenic sarcoma (Fig. 1). Sections from the metastatic lesion invading the small intestine were histologically in- distinguishable from osteogenic sarcoma (Fig. 2). Other metastatic deposits in various organs showed only carci- nomatous component. These findings were suggestive of a primary carcinosarcoma with systemic metastases. Repre- sentative sections were sent to the Armed Forces Institute of Pathology, Washington, DC. The case was reviewed by their Pulmonary and Mediastinal Branch with consultation of the Soft Tissue Branch. Their interpretation was that the sections represented a pulmonary carcinosarcoma based upon the intrabronchial growth pattern and a mixed carci- nomatous and sarcomatous differentiation in the primary and metastatic sites.

DISCUSSION The primary mixed malignancies of the lung are rare

tumors that contain both carcinoma and sarcoma.

Accepted for publication August 5 , 1981. Address reprint requests to Deba P. Sarma, MD, VA Medical Center, 1601 Perdido Street, New Orleans, LA 70146.

Pulmonary Carcinosarcoma 217

Fig. 1. Photomicrograph of the lung tumor showing carcinosarcoma (HE, x 100).

Fig. 2. Photomicrograph of the metastasis in the intestine showing osteosarcoma (HE, X 200).

218 Sarma and Deshotels

Histogenesis of these tumors has been controversial. The- oretical proposals based on evidence from case reports and experimental data have ranged from collision tumors [7], benign sarcomatoid stromal reaction to a carcinoma [8], mesenchymal transformation of primary epithelial malig- nant tumors [9], malignant degeneration of pulmonary ha- martomas [ 101, induction of malignant change in both ep- ithelial and connective tissue elements [ I l l , and dual malignancy arising in primitive pluripotential lung blastema [12]. This last theory was proposed by Spencer [12] to explain the histogenesis of the embryonal type pulmonary carcinosarcoma (pulmonary blastoma). This was based upon the experiments of Waddell [ 131 who felt that the distal portions of lung tissue actually developed from mesenchyme not merely from subdividing into surrounding connective tissue. This process was thought to be similar to renal em- bryogenesis based upon similar histologic appearance of pulmonary blastoma and nephroblastoma (Wilm’s tumor). Data from animal experiments from which this theory was derived were tenuous and were not well accepted as sum- marized by Fung et a1 [14].

Another report demonstrated evidence of a pulmonary blastoma showing a differentiation pattern of a carcinosar- coma [15]. This study lent support for the concept of the pulmonary blastoma as a subtype or a variant of carcino- sarcoma.

Our present case and analysis of other reports indicate that these tumors from the lung and upper respiratory tract represent a heterogeneous population of epithelial tumors that undergo sarcomatous transformation. This process probably occurs through mesenchymal metaplasia. The po- lypoid growth pattern typical of pulmonary carcinosarcoma suggests a relationship to similar tumors of the larynx, esophagus, soft palate, and tongue. Electron microscopic studies of spindle cell carcinomas of the upper respiratory tract have shown that these cells are predominantly of an epithelial nature [16]. Ultrastructural analysis of such a tu- mor by Battifora et a1 [17] showed the tumor cells to be spindle cells with both fibroblastic and epithelial features. They concluded that the tumor cells were epithelial in nature and had undergone metaplasia with collagen production. Oyasu et a1 [18] reported a squamous cell carcinoma as- sociated with an osteoclastoma of the lung indicating that the process represented a derepression of malignant epithe- lial cell potentiality.

Our case of lung tumor with the metastatic mesenteric lesion showing an osteogenic sarcoma pattern supports the theory of a mesenchymal differentiation of epithelial tu- mors.

From the review of literature and our own experience several general features of carcinosarcomas of the respira- tory tract can be elicited. In most reported cases, a prominent intermingling pattern of the neoplastic elements is present. Lymph node metastases appear to be composed primarily of the epithelial component, whereas hematogenously dis-

seminated lesions are more likely the ones to exhibit sar- comatoid features of a variable nature. The pulmonary blas- toma seems to represent a spectral manifestation of carcinosarcomas of the lung, perhaps a dedifferentiation to a more primitive growth pattern, although they may rarely arise from a pulmonary hamartoma.

The favorable prognosis of pulmonary carcinosarcomas relative to carcinomas is probably related to their polypoid endobronchial growth pattern, often central location leading to early obstructive symptoms, providing an opportunity for earlier diagnosis and a greater chance for successful treat- ment. Indeed, lesions that are peripherally located tend to be clinically silent and have a poorer outcome due a longer detection time with greater opportunity for spread.

Careful ultrastructural studies of carcinosarcomas of the respiratory tract are needed to document firmly the process of sarcomatous transformation from malignant epithelial cells. Close follow-up studies are necessary in living patients with such tumors to establish metastatic behavior and ul- timately to determine which pattern or patterns of these difficult neoplasms will respond best to various treatment modalities.

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