Pancoast Syndrome Due to Metastatic Carcinoma from the Uterine Cervix

Pancoast Syndrome Due to Metastatic Carcinoma from the Uterine Cervix

SELEcrED REPORTS Pancoast Syndrome Due to Metastatic Carcinoma from the Uterine Cervix * Cilbert S. Omenn, .\I.D. O O Symptoms of the Pancoast syndro...

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SELEcrED REPORTS Pancoast Syndrome Due to Metastatic Carcinoma from the Uterine Cervix * Cilbert S. Omenn, .\I.D. O O

Symptoms of the Pancoast syndrome appeared in a 45year-old woman 18 months after apparently successful treatment of stage III carcinoma of the cervix uteri. This is believed to be the first report of the presentation of metastatic carcinoma of the cervix as a thoracic inlet tumor. pancoast described in 1924 and in 1932 a total of seven patients having a syndrome of thoracic inlet obstruction due to tumor. I.~ The primary manifestations are pain around the shoulder and down the medial aspect of the arm, Horner's syndrome, atrophy of the small muscles of the hand, and x-ray evidence of a lesion at the extreme apex of the lung with local destruction of ribs or vertebrae. The full clinical syndrome was reported first by Hare in 18:38.:1 Although Pancoast postulated that the tumor arose in an embryonal epithelial rest at the apex of the lung, many cases due to metastatic tumor, as well as local inflammatory and infectious processes, have been recognized. Most tumors have arisen in the pulmonary parenchyma. but others have spread from the stomach, pancreas, kidney, prostate, thymus, breast, bone, thyroid, larynx, and pleura.":" In large series of cases,';" there has been a marked preponderance of men to women (10: 1), mostly attributable to the much higher incidence of carcinoma of the lung in men.? This report records a case of the Pancoast syndrome due to a thoracic inlet tumor metastatic from the uterine cervix.

FIGURE l A. Chest x-ray film four months after onset of right shoulder pain and Horner's syndrome, showing fullness of the right apex. she lacked respiratory symptoms and had a good appetite. Cytologic examination of the sputum was negative. A skeletal x-ray series and a lymphangiogram of lower extremities and aortic areas gave normal findings. She received 5000 r with a 2 \IEV machine via anterior and posterior portholes to the right superior sulcus area. During therapy, she developed severe pain in the 4th thoracic vertebral body and was given an additional 1000 r to that site. However, pain persisted, despite phenol block and then left cordotomy at the CI-2 level. On her final admission in January, 1967, she was emaciated, febrile, and in constant pain. She had right-sided Horner's syndrome, and the right supraclavicular fossa was filled with matted tissue. The right hemi-diaphragm was elevated and immobile, and breath sounds were absent over the right upper lobe posteriorly. The trachea was midline, and there was no adenopathy or organomegaly. The right upper extremity was paralyzed and atrophic, with flexion contractures at the elbow and the shoulder. There was a fluctuant mass in the left thigh, but no calf tenderness, edema, or clubbing. She was alert and oriented, with normal plantar responses. X-ray films showed a right upper lobe infiltrate without new signs of tumor, but with collapse of the third to fifth cervical vertebrae, consistent with right phrenic nerve paralysis (Fig 1B). Her hematocrit was 24 percent, white blood cell count 21,700 with 90 percent neutrophils. The urine contained abundant Gram-negative rods. Despite intravenous fluids and antibiotics appropriate for her urinary tract infection, she became hypotensive and died quietly the next morning. Pneumococci were grown from blood cultures. Autopsy revealed involvement of the uterus, adnexa, bladder, and rectum by dense fibrous adhesions, but no tumor could be found in the peritoneal cavity. The pelvic fibrosis caused bilateral ureteral strictures, with hydronephrosis and chronic pyelonephritis on the left. A few slightly nodular, grey-tan plaques were noted on the diaphragm, several mm in diameter and 1-2 mm in thickness. The pleural spaces contained 50 ml of serous fluid each. There was a dense fibrous adhesion over the apex of the right lung, the posterior surface

CASE REPORT

In April, 1964, a previously well 45-year-old white housewife developed left lumbosacral and left groin pain. Stage III squamous cell carcinoma of the cervix uteri was diagnosed, and she was treated with a total of 7400 r by radium implantation. In January, 1965, she complained of pain in the left costovertebral angle. The left ureter was reimplanted into the bladder, and she had no further evidence of obstruction of the urinary tract. She came to the Massachusetts General Hospital in December, 196.'5, with a three-month history of pain under the right scapula. A tumor of the thoracic inlet was suspected only after silt' developed ptosis of the right eyelid and diminished sweating on the right side of the face. A chest film showed fullness of the right apical area, while the pulmonary parenchyma appeared normal (Fig I A). Because she had smoked two packs of cigarettes per day for 20 years, sht, was thought to have a primary pulmonary neoplasm, but °From the Medical Service, Massachusetts General Hospital, Boston. o 0Prest'ntly at Division of Medical Genetics, University of Washington, Seattle.

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PANCOAST SYNDROME DUE TO METASTASIS FROM UTERINE CERVIX

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nuclei of variable size and chromatin pattern, consistent with metastatic squamous-cell carcinoma of the uterine cervix ( Fig 2). The right upper and middle lobes contained much fibrosis and many neutrophils and macrophages. :\0 tumor cells were found in sections of the pelvic tissues. COl\[},fENT

FIGURE 1B. Chest x-ray film on final admission 12 months later, showing elevation of the right hemi-diaphragrn due to phrenic nerve paralysis and fibrosis and pneumonitis in the right upper lobe. of the right upper lobe, and the upper 10 ern of the lower lobe. The grey-tan, finn tissue filled the thoracic inlet, extended upward around the brachial plexus and vessels to the right upper extremity and side of the neck, surrounded the first rib, occupied the marrow cavity from the fourth thoracic to the third cervical vertebra, and extended within 1 em of the thyroid gland. It was separated from the skin by several mm of adipose tissue. The right upper lobe contained an area of consolidation. There was no right ventricular hypertrophy. The Huctuant mass in the thigh contained cloudy tan Huid and a wall of cauliflower-like, nodular, grey-tan tissue. The brain was normal, but the cervical spinal cord was sclerotic at the cordotomy site. Microscopically, the right thoracic apical and inlet areas, vertebral marrow cavities, and left thigh mass consisted of dense fibrous tissue infiltrated by nests of large, polyhedral tumor cells with abundant. eosinophilic cytoplasm and oval

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l"IGURE 2. Fibrous tissue infiltrated with nests of large, polyhedral tumor cells and with scattered neutrophils in the right thoracic inlet (hematoxylin and eosin, X 22.'5).

CHEST, VOL. 60, NO.3, SEPTEMBER 1971

Aching pain in the interscapular, scapular, or pectoral areas is the initial and dominant complaint in almost all cases of thoracic inlet tumors. Early diagnosis requires careful comparison of the apical areas on chest x-ray films for unilateral opacification and for destruction of portions of ribs or vertebral processes. These tumors must be considered together with benign skeletal and inflammatory conditions in the differential diagnosis of pain in the region of the shoulder, and special lordotic views of the apical areas must be obtained promptly. The typical features of the Pancoast syndrome are late signs due to involvement of the inferior trunk of the brachial plexus (C8,Tl,T2, innervating the medial aspect of the upper extremity and the intrinsic muscles of the hand), inferior cervical ganglion (Horner's syndrome), phrenic or recurrent laryngeal nerves, spinal cord, and superior vena cava. Respiratory symptoms are infrequent, and pleural effusions are rare.":" When the diagnosis was made and radiotherapy initiated in our patient, involvement of essential structures was well-advanced. Her rapidly progressive course of illness is characteristic for these tumors, despite occasional success with radiation and radical surgery.v" The sites of tumor foci are compatible with hematogenous metastasis from the cervix to the thoracic inlet and to the thigh, with extension of the apical tumor and seeding of the diaphragm to produce the clinical and pathologic findings. There was no evidence for a second primary neoplasm. It is surprising that carcinoma of the cervix has not been associated before with the Pancoast syndrome, since the pulmonary parenchyma is a favored site for distant metastasis of this very frequently-occurring tumor.IO-I~ Of patients with carcinoma of the cervix that appears to be stage Ill, as in this case, 20 percent eventually develop distant metastases, with the lung the most common site for single-organ involvement.t-' In many respects, this patient's history resembles closely those of the two women patients among Pancoast's original seven cases (cases 6 and i).~ Each had required radiotherapy for carcinoma of the cervix within three years of the appearance of the thoracic inlet syndrome. The apical tumor was considered to be unrelated only because there was no clinical evidence of recurrent tumor in the pelvis or in other sites. No biopsy or autopsy material was obtained in either case. Recognizing that many other metastatic tumors have produced the typical Pancoast syndrome, we believe that the apical tumors in Pancoast's cases of women were metastatic carcinoma of the cervix. These cases of unusual patterns of metastasis expose our limited understanding of the biologic behavior of even the common neoplasms and of the host interaction. However, on the practical clinical level, these cases serve

SMYTH ET AL

270 as an example for the .dictum that unusual manifestations of common diseases always must be considered in the differential diagnosis of rare syndromes. ACK;,\OWLEJ)C\IE;,\T: The assistance of the late Dale Wenlund, \I.D., of tilt' Radiology Department, and of Hoger A. Williams, \I.D., of the Pathology Department, is gratefully acknowledged. REFEHE;\;CES

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Pancoast H K: Importance of careful roentgen-ray investigations of apical chest tumors. JA\fA 83: 1408-141 I, 1924 Pancoast HK: Superior pulmonary sulcus tumor: Tumor characterized hy pain, Horner's syndrome, destruction of bone and atrophy of hand muscles. JA\fA 99: 1391-1396, 1932 Hare ES: Tumor involving certain nerves. London \1ed Cazettt- 23: 16-18, 1838 Browder J, DeVeer J: Varied pathologic basis for symptomatology produced by tumor in the region of the pulmonary ape-x and nu-diastinurn. Amer J Cancer 24:507.521, 193.5 \forris JH, Harken DE: The superior pulmonary sulcus "tumor of Pancoast" in relation to Hare's syndrome. Ann Surg 112: 1-21, 1940 Herbut PA, \Vatson jS: Tumor of the thoracic inlet producing the Pancoast syndrome: a report of 17 cases and a review of the literature. Arch Path 42:88-103, 1946 Hepper :'\CC, et al: Thoracic inlet tumors. Ann Intern \fed 64 :979-989, 1966 Shaw HH, Paulson DC, Kee jL, jr: Treatment of the superior sulcus tumor by irradiation followed by resection. Ann Surt; !.'>4:29-40, Hl6! \fcCoon DC: Trunscervical technic for removal of specimen from superior sulcus tumor for pathologic study. Ann Sun; \.'59:407-410, 1964 Craham jB, Sotto LSj, Paloucek FP: Carcinoma of the Cervix. Philadelphia, W. B. Saunders lH62, pp 64-79 Ingersoll F\I, Ulfelder H: Pelvic exenteration for carcinoma of the cervix. New Eng J \f(·d 274:648-6.51, 1966 Carlson V, Delclos L, Fletcher GH: Distant metastases in squamous-cell carcinoma of the uterine cervix. Radiology HIU)()\ - 9(;f), 19(;(;

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u lm o n a ry asbestosis is an occupational disease! which can affect any person working in a factory or industry dealing with asbestos or its products, or living in the vicinity of such an establishment. One of the more common forms of pneumoconiosis, asbestosis, is a slowly progressive disease of the lungs and pleura, often appearing as long as 20 to 40 years after exposure, making diagnosis difficult in sporadic cases. In the last decade interest in this condition has gained new momentum because of its possible relationship to pleural and peritoneal mesotheliorna-o--a suspicion that has been borne out by experimental reproduction of this tumor in lower animals:l-and also because of its close association with bronchogenic and gastric carcinoma.':" In a community hospital serving an urban population without a nearby factory or industry using asbestos, cases are seen infrequently and the diagnosis is often not considered. Because of the long interval between exposure to asbestos and the clinical onset of the disease, the occupational history, so important to the diagnosis, may not be obtained. CASE REPORT

A 68-year-old retired government analyst was admitted to the Washington Hospital Center on june 6, 1969, because of cough, weakness and unexplained pleural effusion. Since October 1968, he had noted night sweats. In March 1969, he developed a cough with production of greenish sputum, He had become increasingly short of breath over a period of several months. I n tilt' past, he had sustained a head injury at age 1.5 and had suffered from severe headaches thereafter. He had had scarlet feve-r at age 17, an operation on his nose at age 21, and varicose vein surgery at age 47. He had suffered a suharachnoid hemorrhage in \larch of 1963. A full neurologic investi-

Hc-priut requests: Dr. Omenn, University Hospital, Seattle 9HIO.'5

Pulmonary Asbestosis* Nicholas P. D. Smyth, M.D., F.C.C.P.;oo Nelson G. Goodman, .\t.D.;t Asit P. Basu, M.D.;tt and John M. Keshishian, .\t.D., F.C.C.P.*

We report a case of asbestosis in a 63-year-old man, diagnosed 45 years after exposure to asbestos. The case is unusual in the finding of hemorrhagic pleural effusion without associated malignancy. o From

the Departments of Surgery and \Iedicine, Washington Hospital Cente-r, Washington, D.C . •• Associate Clinical Professor of Surgery, Ceorge Washington Universitv School of \ledicilH·. tClinical Instrildor in \Iedicillt', C,-mge Washington University School of Medic-im-. ttSurgical Iksearch Fellow, Washinuton Hospital Center. *Assistant Clinical Professor of Surgery, Ceorge Washington University School of Medicine.

FIGUHE 1. Chest x-ray film (1964) showing extensive pleural calcification most marked in the left mediastinal pleura.

CHEST, VOL. 60, NO.3, SEPTEMBER 1971