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April 1980, Volume 30, Issue 4

Case Reports

CARCINOSARCOMA OF THE LUNG REPORT OF A CASE AND REVIEW OF THE LITERATURE

Shamimul Haque  ( Department of Pathology, Monmouth Medical Centre, Long Branch, New Jersey, U.S.A. Present Address: Dept. of Anatomy, Ayub Medical College, P.O. Jhangi, Abbottabad, Hazara, N.W.F.P. )

Abstract

Pulmonary carcinosarcoma is a rare tumor composed of both carcinomatous and sarcomatous elements. Thirty cases of pulmonary carcinosarcoma have thus far been reported. An additional case in a 57 year old female with a carcinosarcoma in the left lung is reported. This case is unusual because it presented with multiple carcinomatous (squamous cell and adenocarcinoma) and sarcomatous (chondrosarcoma and undifferentiated sarcoma) components. In addition, this patient had a right pneumonectomy for an adenocarcinoma of the lung 7 years before. This is the first reported case of a second primary pulmonary carcinoma, associated with pulmonary carcinosarcoma.
From the analysis of published cases, it is apparent that there are two distinct types of pulmonary carcinosarcomas. In one type, the tumor is peripheral, and in the other type, the tumor is central endobronchial with a better prognosis. The clinical history and roentgenography are not particularly characteristic for this tumor. Pulmonary resection when feasible is the effective form of therapy.

Introduction

Carcinosarcomas (mixed malignant tumour) are rare malignant neoplasms that contain simu-tancosly intermixed elements of carcinoma and sarcoma. They have arisen, in decreasing order of frequency, in the uterus, hypopharynx, esophagus and lungs (Saphir and Vass, 1938). Since the original description of pulmonary carcinosarcoma by Kika (Bergmann 1951) in 1908, 30 cases have been reported.
The purpose of this paper is to report an additional case of pulmonary carcinosarcoma and to review in some detail the published experience in 30 cases of pulmonary carcinosarcoma.

Case Report

This patient, a 57 year old white female, was admitted to the Monmouth Medical Centre on September 27, 1974 because of progressively-increasing shortness of breath. In May, 1967 a right pneumonectomy had been performed at another hospital for carcinoma of the lung. Review of the slides confirmed the original histologic diagnosis of adenocarcinoma with anaplasia (Fig. 1).

In the slides reviewed, the lesion appeared to be moderately differentiated adenocarcinoma, and there was no evidence of a sarcomatous component. There were no metastases in hilar lymph nodes. She did not receive any radiation or chemotherapy at that time. In April, 1973 a routine x-ray of the chest revealed a left hilar mass which was thought to be recurrence and/or metastasis from the primary tumour removed in 1967. Radiation therapy to the left lung was begun. After the patient had received 1,000 rads, she refused to take further radiation therapy and was therefore placed on chemotherapy with Cytoxin, Oncovin and Prednisone for 4 months without much improvement. During the last year she had several admissions to Monmouth Medical Centre for respiratory failure and con­gestive heart failure which was treated with Digitalis and Prednisone.
On physical examination the patient appeared chronically ill, she was drowsy and somewhat uncooperative. There was no rash or lymphaden-opathy. Breath sounds were absent on the right side and were decreased on the left side with a few moist rales at the base. The heart was not enlarged. The liver edge was barely palpable, the spleen was not enlarged and there were no abdominal masses. Neurological examination was negative.
X-ray of the chest revealed a mass lesion extending from the right midline to the lateral chest wall on the left. An EKG showed changes of right ventricular hypertrophy.
The hematocrit was 45.8 per-cent; the white cell count was 12,000 with 85 per-cent neutrophils. The urea nitrogen was 15 mg/dl, the sodium was 139 meq/1; the potassium was 3.9 meq/1 and the chloride 90 meq/1. The CO2 content was 32.8 meq/1; the pH was 7.16 and the PC02 was 84 mm Hg
During her stay in the hospital, the patient was afebrile. She became increasing unresponsive. She expired on the second hospital day in respiratory failure. An autopsy was performed
Pathology
The left lung weighed 840 gms with a tumour mass in the upper lobe measuring 14 x 13 x 12 cm. The pleural surface of the lung was intact, there was no direct invasion of the chest wall or thoracic cage (Fig. 2).

The tumor had an endobronchial component in the form of finger-like projections growing in the lumen of the main stem bronchus (Fig. 3).

The upper lobe was almost completely replaced by a firm tumor. When cut, there was a variegated appearance with areas of hemorrhagic necrosis in a tan parenchyma which contained small nodular, grey, glistening, firm areas (Fig. 4).

Microscopically the bulk of the tumour was composed of round spindle shaped undifferentiated sarcoma (Fig. 5).

Scattered in it were focal areas which had an organoid appearance reminiscent of carcinoid tumour (Fig. 6).

Occasional multinucleated giant cells resembling rhabdomyoblasts were seen, but no cross stria-tions could be demonstrated (Fig. 7).

Nodular masses of well to moderately chondrosarcoma was also present (Fig. 8 and 9).


The carcinomatous element was composed ofsquamous cell carcinoma with keratin pearl formation (Fig. 10)

as well as foci of adenopapil-lary carcinoma (Fig. 11).

There were no pleural, chest wall or disdant metastases, but the tumor did show vascular (Fig. 12)

and perineural lymphatic invasion (Fig. 13).


REVIEW OF LITERATURE
A summary of 30 cases reported in literature is presented in the accompanying table.


Clinical Features
Of the 30 cases with carcinosarcoma reported in the literature, there were 24 men and 6 women with M:F ratio of 4:1. The age ranged from 35 years to 77 years with the highest incidence in the sixth and seventh decades. Twenty patients had symptoms referable to the respiratory tract. 16 patients complained of cough; 8 gave a history of hemoptysis; 8 of chest pain; 3 of dyspnea and 2 of hoarseness. Significant weight loss was observed in 8 patients. The case reported by Weber (Bergmann et al 1951) presented with anorexia, abdominal pain and vomiting. The clinical picture was explained by finding numerous gastrointestinal metasteses. Ogawa's (Bergmann at al 1951) case presented with malaise and dull pain in the shoulder with radiation to the back. Three of the 9 cases reported by Stackhouse et al (1969) were asymptomatic; a mass was found on routine x-ray of the chest. X-ray evidence of bronchial obstruction with atelectasis was seen, in 8 cases. A mass was seen on x-ray in 18 cases. One case reported by Stackhouse et al (1969) had a lymphosarccoma nine years prior to pulmonary neoplasm and had been given radia­tion therapy.
Gross Pathology
The tumours varied in size; the smallest was 1 cm and the largest 14 cm in diameter (Bergmann et al., 1951). Three major forms of pulmonary carcinosarcomas were seen: (a) solely endobronchial; (b) solely peripheral or parenchymatous; (c) endobronchial with parenchymatous component. Of the 30 cases reported in literature, 10 were solely endobronchial; 13 were only parenchymatous (this includes 7 cases which were diagnosed at autopsy and were extensive with multiple distant metastases); 6 cases had both endobronchial and parenchymatous component.
Gross features of these tumours such as colour, and consistency were nonspecific and varied considerably. All extrabronchial masses were moderately firm, homogenous, multinodular circumscribed lesions which were pinkish or yellow-grey (Bergmann et al., 1951; Stackhouse et al., 1969). The intrabronchial components were polypoid and similar to the under-lying parenchy mal component (Bergmann et al., 1951; Stack-house et al., 1969).
Microscopic Description
A. Carcinoma
The most common carcinomatous element was epidermoid or squamous cell carcinoma which was seen in 20 cases. Adenocarcinoma was present in 5 cases. Adenocarcinoma with undif­ferentiated carcinoma was seen in 2 cases. Undifferentiated Carcinoma; squamous cell with undifferentiated carcinoma; and adenocarcinoma with squamous and undifferentiated components were observed in 1 case each.
B. Sarcoma
The most common sacromatous component observed was a spindle cell or fibresarcoma which was seen in 25 cases. In 1 case the sarcomatous element was composed of elements of fibrosar­coma, chondrosarcoma and osteosarcoma. Fibrosarcoma and osteosarcoma were seen in 2 cases. Chondrosarcoma and osteosarcoma; and fibrosarcoma and chondrosarcoma were found in one case each.
Metastases
Bergmann et al reviewed the literature in 1951 and found 7 examples of such lesions. All were diagnosed at autopsy. All except 1 showed distant metastases; the adrenals and the kidneys were the most common sites of metastases. Both malignant elements were present in 2 cases and the rest of the cases showed one malignant element. In Stackhouse's (1969) series, regional nodular metastases were noted in 5 of 8 patients who underwent operation. Both malignant ele­ments were present in 3 patients; carcinoma only or sarcoma only was noted in 2 others by direct extension, and distant metastases were observed in 2 cases.
Survival
Known survival varied from 18 months to 6 years in 6 patients. These were patients last seen at 18 months (Prive et al., 1961); 19 months (2 patients) (Bergmann et al., 1951; Moore, 1961); 3 years (Taylor et al., 1952); 3 years 8 months (Stackhouse et al., 1969) and 6 years (Bergmann et al., 1951) without clinical evidence of recurrence. It is of note that predominantly endobronchial neoplasms were found in the survivors. The experience is similar to the pedunculated carcinosarcoma arising in the esophagus and hypo-pharynx.

Discussion

The separate classification of carcinosarcoma in any organ system is a debated issue. Willis (1967) defines carcinosarcomas as a simultaneously malignant neoplasia in two distinct tissues; an epithelial tissue and a non-epithelial tissue; or a consequent sarcomatous change in the stroma of a carcinoma. The fortuitous development of two separate tumours, a carcinoma and a sarcoma in continuity (\'collison tumour\') should not be called carcinosarcoma. Saphir et al (1938) reviewed the subject and found 153 examples of carcinosarcomas of all sites. They accepted 3 or 4 cases as being carcinosarcomas and they expressed the opinion that all the other cases were in reality examples of morphological capabilities of one type of cell. In their analysis they found many complicating factors which played a role in the alteration of the fundamental histologic appearance of the tumour, such as: (a) variation of carcinoma cells, some of which can assume spindle shapes and may be interpreted as cells of spindle cell sarcoma, a feature which is particularly true of squamous cell carcinoma; (b) marked anaplasia of carcinoma cells; (c) chronic inflammation which either leads to morphologic change of tumour cells or provokes much connective tissue response which may be regarded as a part of malignant connective tissue tumour; (d) invasion of benign connective tissue tumour; and (e) sarcomas which had invaded normal or metaplastic epithelial structures.
However, carcinosarcoma, although rare, is a distinct pathologic entity. This is supported by experimental studies of transplants of mammary carcinoma in mice (Stewart et al., 1959). While transplanting pulmonary carcinoma subcutaneously, Stewart and associates noted the emergence first of carcinoma and then fibrosarcoma. On the basis of x-ray induced ovarian tumors in mice, Aykan (1956) concluded that the two kinds of elements undergo neoplastic transformation simultaneously and independently. There are various conditions which may simulate carcinosarcoma; most of which are merely carcinoma with an anaplastic epithelial component mimicking malignant mesodermal stroma. However, the presence of dense reticulin, malignant cartilage and bone is helpful in differentiating the two lesions. Sarcomatous development in a hamartoma can simulate carcinosarcoma. Cavin et al (1958) reported a case of leiomyosarcoma arising in a harmartoma. In none of the 31 cases including our own, was there any evidence of pre-existing hamartoma. Another suggestion that these tumours arise in teratomas is also acceptable; in a teratoma a variety of hetrogeneous somatic elements are arranged in a more or less jumbled or haphazard fashion (Willis, 1967). This pattern is not seen in any of the carcinosarcomas of the lung. Pulmonary blastoma is a distinct entity and morphologically consists of well differentiated adenocarcinoma in a stroma of immature or undifferentiated sarcoma (Minken et al., 1968; Bauermeister et al., 1966).
From an analysis of 31 cases including our own, it is apparent that there are two distinct types of pulmonary carcinosarcomas. In one type the tumour is peripheral or parenchymatous. It may attain considerable size prior to detection and has a tendency towards direct invasion of the chest wall and mediastinal structures. There is also a tendency towards distant metastases and the prognosis is poor. The second type of tumour is more centrally located. It occurs as a pedunculated endobronchial growth. The prognosis with this type of tumor is surprisingly good. Pulmonary resection when feasible is the effective form of therapy.
The case reported in this paper has certain unusual features. The tumour presented with multiple carcinomatous (squamous cell and adenocarcinoma) and sarcomatous (chondrosarcoma and undifferentiated sarcoma) elements. As far as we know, there are 5 cases in the literature which presented in a similar fashion (Moore, 1961; Prive et al., 1961; Stackhouse et al., 1969). Another unusual feature of this case is the occurrence of an adenocarcinoma in the other lung 7 years before. The subject of two histologically different primary carcinomas of the lung has been reviewed by Mobley and Martinez (1968). They found 33 such cases in the literature and reported an additional case. None of their cases were associated with carcinosarcoma. To our knowledge, this is the first reported case of carcinosarcoma associated with another histologically different primary carcinoma of the lung.

References

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