Pulmonary Blastoma; Longterm Survival of Juvenile Patient

Pulmonary Blastoma; Longterm Survival of Juvenile Patient

436 10 11 12 13 14 15 IVERSON, STRAEHLEY microembolism in experimental shock. An electron microscopic study. Am J Path 52:391-400, 1968 Schramel R,...

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10 11 12 13 14 15

IVERSON, STRAEHLEY

microembolism in experimental shock. An electron microscopic study. Am J Path 52:391-400, 1968 Schramel R, Schmidt F, Davis F, et al: Pulmonary lesions produced by prolonged partial perfusion. Surgery 54:224231, 1963 Rosky LP, Rodman T: Medical progress: Medical aspects of open-heart surgery. N Engl J Med 274:833-840, 1966 Pulmonary microembolism, Leading articles, Lancet 1:429-431, 1967 Brain MC: Medical intelligence: Microangiopathic hemolytic anemia. N Engl J Med 281 :833-835, 1969 Glueck HI, Hermann LG: Cold-precipitable fibrinogen, Cryofibrinogen. Arch Int Med 113:748-757, 1964 Rodriguez-Erdmann F: Bleeding due to increased intravascular blood coagulation. N Engl J Med 273:1370-1378, 1965

Pulmonary Blastoma; Longterm Survival of Juvenile Patient Ronald E. Iverson, M.D.o and Clifford J. Straehley, M.D.,

F.C.C.P.oo

FIGURE 1. PA view of chest in 11-year-old boy with 8 x 8 x 10 em mass in lower lobe.

An ll-year-old boy (at the time of initial treatment) has survived eight years, free of recurrence after left lower lobectomy for pulmonary blastoma. Pulmonary blastoma is a distinct subtype of pulmonary malignancy not be confused with carcinosarcoma. Its distinguishing features are gland-like structures lined by nonciliated epithelium with a surrounding stroma resembling mesenchyma. Three of the 19 known are living five or more years after surgical treatment.

P

ulmonary blastoma is an exceedingly rare primary neoplasm of the lung.' It has also been called a pulmonary embryoma because of its' close histologic resemblance to fetal lung. Its distinguishing features are gland-like structures lined by nonciliated epithelium with a surrounding stroma resembling mesenchyma. The neoplasm is so rare that relatively few cases are reported, and the natural history of the lesion is not clearly defined. Therefore, an additional case of pulmonary blastoma, occurring in an ll-year-old boy who has survived eight years following surgery without evident residual or recurrent disease, is worthy of report. CASE REPORT

An ll-year-old boy of Japanese ancestry was admitted to the Honolulu Kaiser Foundation Hospital in April, 1964. A week earlier, a brisk episode of apparent bleeding from the nasal pharynx had occurred, but careful otolaryngologic examination revealed no site of bleeding. His hemoglobin was 10 gm. Two days later, hemoptysis was suspected as the °Presently Senior Resident in Surgery, Stanford University Medical School, Stanford, California. oOChief, Department of Surgery, Kaiser Foundation Hospital, Honolulu; Associate Clinical Professor of Surgery, University of Hawaii Medical School. Reprint requests: Dr. Straehley, 1697 Ala Moana Blvd, Hono-

lulu, Hawaii 96815

FIGURE 2. Lateral view of chest-confirming the lower lobe lesion.

CHEST, VOL. 63, NO.3, MARCH, 1973

PULMONARY BLASTOMA

437

caus e of the blood loss, and x-ray film of the chest was taken. This showed an 8 x 8 x 10 cm mass in the left lower lung field ( Fig 1 and 2 ). It had sharp marg ins with no evidence of surround ing inflammation. X-ra y examination of the skull, sinuses and facial bones gave norm al findings. Intravenous pyelogram was interpreted at th at time as normal. Results of an upper GI series were norm al. Th e patient was placed on iron by mouth, and after a period of two weeks, was read mitte d for surgery. Physical examination revealed a welldevel oped, well-nourished boy who showed no evidence of tall and weighed 78 the onset of puberty. He was 4 '!5~" pounds. Examination of the lungs gave normal findings. Th ere was a grade 1 systolic murmur heard at the apex of the heart . There was no palpable organ or mass in the abdomen. The testes were infantile in size and descended. Left thoracotomy was performed , A mass varying in diameter between 10 em and 15 em virtu ally filled the left lower lobe. There was no evidence of extensi on of this mass to the chest wall or pericardium and no implants on the pleura. Th ere were no obviously enlarged mediastinal nodes. Left lower lobectomy was performed. Frozen section was reported as being consistent with an embryonal carcinoma. Therefore, prior to closure of the chest, the left kidney was palpated through an intact diaphragm. It felt normal. An IVP was rep eated with a nephrotornograrn on the sixth postoperative day. Thi s showed a prominence of the upper inner margin of the left kidney. The pennanent section of the lung tum or (Fig 3 and 4 ) was interpreted as an embryona l adenomyosarcoma. The kidney was suggested as a possible primary site. The urologic consultant concurred, and he perform ed a transabdominal FIGURE 4. Similar area. Note the stratification and mild hyperchromatism of columnar cells (H and E X 125 ).

FIGURE 3. Margin of the lesion showing complex and pleoand plump stromal cells with lar ge dark nuclei (H and E X 75) .

CHEST, VOL. 63, NO.3, MARCH, 1973

exploration of both kidneys ten days after the first operation. Th is revealed both kidneys to be normal except for a slight, pers istent fetal lobulation of the upper pole of the left kidney. Careful exploration of the remainder of the abdomen disclosed no other pathology. The patient did well postoperatively and was discharged seven day s after the second operation. Eight years later he has no evidence of a recurrence of his tumor. Tissue was sent to a' number of other pathologists. Dr. Fred W . Stewart at Memorial Center for Cancer and Allied Diseases in New York City was of the opinion that this was .. 'e mbryonal' hamartomatous lung cancer; and although, naturally, I can prove neither the correctness of this diagnosis nor my feelings that the case outcome may be more favorable than one might expect, I do think that both of these guesses are probably correct." Dr. Averill Liebow, then at Yale Unive rsity stated, "I believ e this represents a primary lung tum or of the mucoepidermoid group. It is noteworthy that some of these mucoepidermoid tumors of the lung have occurred in very young individuals, although the present case is the youngest in my experience. The epithelial component appears definitely malignant and 1 believe the prognosis should be guarded." Dr. Joe M. Bloomberg at Armed Forces Inst itut e of Pathology stated , "the mixed pattern of epitheliallined tubules, the sarcoma-like stroma of plump spindle cells and collagen and solid undifferentiated nest of polyhedral or rounded cells lying both within and outside the tubules and in places appearing to be transitional between the tubular epithelium and the spindle components is strongly suggestive of the rare pulmonary tumor named pulmonary blastoma by Spencer." Dr. E. D. Peasley at Oteen, North Carolina,

438

IVERSON, STRAEHLEY Table I---€linical and Patholo«ic DaJa in Nineteen Ctue. 01 Pulmonary Bla.,oma

CaseNo.

Barnard' 1952 2 Peabody> 1959

Sex Age Symptoma F

40 Cough

Bronchoscopy Negative

M 74 Hemoptysis, Blood seen chest pain LUL orifice

3 Campesi and M 36 Fever, cough Sommariva, 1961 cited by Souza' M 59 Hemoptysis, 4 Spencer1961 shortness of breath

Not done

Chest Film

Treatment

Pathology

RML mll88

Pneumonectomy

9xllcm hilarmll88

Left upper lobectomy

12 X 7 X 7 em solid withnecrosis and hemorrhage 8 em solid mass withina pulmonary cavity or capsule; regional lymph nodesinvolved

Left mid-lung mass

Wedge resection LUL

Partial Left hilar oocIusion, mass LLL bronchus

Left pneumonectomy

5 Spencer1961

M 55 Diplopia; right hemiparesis

Not reported

Not reported

None

6 Spencer1961

F

Negative

LLLmass

Segmental resection

7 Souza· 1964

M 48 Hemoptysis

Negative

9 cm left hilar mll88

Left pneumonectomy

8 Cited by Souza· 1964

M 50

Left lower lobectomy

9 Parker' 1966

M 68 "Cigarette cough"

Retraction LUL

1.7 x 1.5 x 1.5 cm solitary nodule LLL 5 em cavitating mll88 LUL

F

External 5cm mass to compression left hilum LUL bronchus

10 Parker' 1966

19 None

?

33 None

11 Cited by Parker' ? ? 12 BauermeisteJ4 M 59 Hemoptysis 1966

13 Stackhouse' 1969 14 Stackhouse' 1969 15 Stackhouse' 1969 16 Stackhouse' 1969

F

Blood in bronchus PB SegRLL

33 Cough

?

Multiple masses relat. 24 yr history of lesions LULmass

M 68 Cough

?

LULmass

F

32 Cough

?

Right lung multiple lesions

M 60 Cough

?

RMLmass

Alive and no evidence of disease 9 years postoperation Diedof bleeding duodenal ulcer. Autopsy: widespread metastases to retroperitoneal and mesenteric nodes, liver, pancreas, kidneys, adrenal glands Hard rubberymass, Died3 monthslater, cachexia; no autopsy 5 X 6cm LUL

4 X 3.5em solid massin superior segment of LLL; invation LUL bronchus and regional lymph nodes 5.0 X 6 em mass in RUL with metastasisin left cerebral hemisphere; regional nodesnot involved Smooth, gray-white 3.0cm. mass surrounded by compressed tissue, LLL 10 X 7 X 7 em mass in LUL; discretely enlarged lymph nodes, not involved by tumor ?

Left pneumonectomy

5 em cavitating subpleural tumor, hilar nodesinvolved Left 5x4x4cm pneumonectomy extrabronchial and chest wall central mass left excision; radiation lung; nodes negative; evident vascular therapy invasion ?

RL lobectomy

Left pneumonectomy Left pneumonectomy Inoperable; cobalt-60; nitrogen mustard Right middle and partial lower lobectomy

Result

?

Multiple nodules with hemorrhage; four different patterns 5x5x3cm

Empyema; death 5 months postoperation; no recurrence found at autopsy Died5 days after admission; RUL tumor with cerebral metastasis at autopsy Well 1 year later

Well 24 yearslater

?

Died 7 months postopertion; widespread metastatic disease Died widespread metastatic disease 11 years post operation

Well immediately postoperation

5x4x4cm with a cavity

Died I1 years postoperation Died 6 months postoperation Died 7 monthsafter diagnosis

5x4x4cm mass

Alive 1 year postoperation

Continued on page 439

CHEST, VOL. 63, NO. 3, MARCH, 1973

439

PULMONARY BLASTOMA Table l----eontinued Case No.

Sex Age Sumptoms

17 Naaari-

M

18 Lewis-

F

19 Presentreport

M

Bronchoscopy

15 Chest pain

Not done

15 Unknown

Not done

Epistaxis

Not done

and SOB

mos. II

Chest Film Large mass occupying 2/3 of right hemithorax LLL pneumonia and pleural effusion

Veterans Administration Hospital stated, "there is no doubt about this being a pulmonary blastoma. The tumor is extremely well differentiated and shows relatively little evidence of microscopic malignancy." SUMMARY

OF

KNOWN CASES

Previously reported cases (17) appear in Table 1. We have added our report and a personal communication of a patient seen by Dr. J. E. Lewis." Dr. Lewis' patient, a 15-month-old girl, is the 19th occurrence and is the only known case of pulmonary blastoma in an individual in the first decade of life. DISCUSSION

This rarest of pulmonary tumors continues to cause confusion. The diagnosis of the tumor in our case as an embryonal adenomyosarcoma, possibly metastatic, and a questionable IVP finding led to our patient having an exploratory laparotomy after his lung resection. Confusion in regard to pulmonary blastoma results from the further fact that some pathologists have felt it is a carcinosarcoma. Stackhouse et aJ7 in comparing nine cases of carcinosarcoma and four cases of pulmonary blastema seen at the Mayo Clinic, concluded that the pulmonary blastoma is a "distinct sub-type of mixed malignancy." The interpretation of pulmonary blastoma as a unique tumor is in agreement with the other--" opinions recently presented. Thus, there should no longer be serious doubt concerning the classification of this tumor. The question of histogenesis remains unanswered, although Spencer'<'" has offered the explanation that pulmonary blastoma arises from a primitive pulmonary mesodermal anlage. The primitive cell would then form the epithelial and mesenchymal elements of which this mixed malignancy is composed. The question of prognosis for survival of patients with this tumor cannot be answered, due to the small number of cases. Three patients have survived without disease over five years (15, 9 and 8 years, respectively). A fiveyear survival may not be significant with this malignancy since one patient (No. 13) first had a recurrence after ten years and she died one year later. Another patient (No. 10) died 11 years postoperation with the first

CHEST, VOL. 63, NO.3, MARCH, 1973

Treatment Right pneumonectomy Left pneumonectomy Left lower lobectomy

Pathology

Result

Well 2 yearslater Soft cystic 18 cm mass. UnditIerentiated mesenchymal cells-uniform Undifferentiated Died generalized embryoma carcinoma metastases 3 months postoperation Alive and well 8 years lOxlOxl6cm massin LLL; mixed postoperation pattern of epitheleal lined tubulesand sarcoma-like stroma

recurrence two years after her primary resection. Also, Bauermeister et ale reported a patient with a gradually increasing and spreading pulmonary tumor over 24 years. This proved to be a pulmonary blastoma when finally biopsied after the 24 years of observation. It must also be noted that eight of the 19 patients died within one year with metastases present. The best method of treatment is not known, but the results of surgical excision compare favorably with the surgical treatment of other pulmonary malignancies. 11 Parker et al 5 report the use of radiation therapy in one patient (No. 10) who developed several nodules in the right lung, two years following a left pneumonoectomy for pulmonary blastoma. They disappeared completely with radiation. Her next recurrence was eight years later, once again in the right lung, and she died one year following this with evidence of a superior vena caval obstruction. Patient IS was inoperable and was treated with cobalt-60 and nitrogen mustard. This patient died seven months later of the disease. These two cases where radiation therapy has been used do not answer the question of its value in treatment of pulmonary blastoma, and further evaluation is needed. CONCLUSIONS

Lack of familiarity still causes errors in identification of pulmonary blastoma, although this should decrease as more cases are reported. It is now a well-described tumor type.!? Positive identification by pathologists will help the surgeon avoid unnecessary surgery such as the abdominal exploration performed in the authors' case. It will enable the surgeon to treat the primary lesion more appropriately. At present it seems that an aggressive surgical approach to the primary lesion is indicated. There are certain questions concerning this rarest of known pulmonary tumors which have been answered. It is a distinct subtype of mixed malignancy not to be confused with carcinosarcoma. The prognosis is poor, although this statement must be modified by the fact that three of the 19 known cases of pulmonary blastoma are alive and well over five years postoperation. REFERENCES

1 Barnard WG: Embryoma of lung. Thorax 7:299,1952

440 2 Peabody CN: Carcinosarcoma of lung of peripheral origin. J Thorac Cardiovasc Surg 39:766, 1959 3 Souza RC, Peasley ED, Takaro T: Pulmonary blastoma. Ann Thor Surg 1:259, 1965 4 Spencer H: Pulmonary blastoma. J Path Bact 82:161, 19& 5 Parker JC jr, Payne WS, Woolner LB: Pulmonary blastoma (embryoma}: Report of 2 cases. J Thorac Cardiovase Surg 51:694,1966 6 Bauermeister DE, Jennings ER, Beland AH, et a1: Pulmonary blastoma a fonn of carcinosarcoma. Am J Clin Path 46:322, 1966 7 Stackhouse EM, Harrison EG, Ellis FH: Primary mixed malignancies of lung: carcinosarcoma and blastoma. 57: 285, 1969 8 Nazari A, Amer-Mokri E, Sarram A, et al: Pulmonary blastoma. Chest 60: 187, 1971 9 Lewis JE Jr: Personal communication 10 Spencer H: Pulmonary blastoma (embryoma). In Pathology of Lung. New York, MacMillan, 1968,985-986 11 Galofre M, Payne WS, Woolner LB, et al: Pathologic classification and surgical treatment of bronchogenic carcinoma. Surg Gynec Obst 119:51, 1964

BURG ET AL CASE REPORT

A 48-year-old housewife with status anginosus was admitted to the University of Oregon Medical School Hospital in June, 1971. For five weeks she had experienced increasing cardiac pain, initially responsive to nitroglycerin. There had been no previous chest pain or evidence of myocardial infarction. She never had rheumatic fever, and a heart murmur had not been heard before. Her blood pressure was 115/70 mm Hg, The heart was not PHOHO

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Disappearance of Coronary Artery Stenosis Murmur after Aortocoronary Bypass* John R. Burg, M.D.; Kathleen A. Weaver, M.D.; Thomas Bussell II, M.D.; and Donald G. Kassebaum, M.D.

A woman with a diastolic coronary artery stenosis murmur which disappeared immediately after aortocoronary bypass surgery is reported. Two months postoperation, the murmur reappeared coincidental with acute anteroseptal myocardial infarction, presumably secondary to occlusion of the bypass graft. Ten months following surgery, when coronary arteriography showed occlusion of the bypass graft and the affected coronary artery, the murmur was no longer present.

A

diastolic murmur due to stenosis of a coronary artery is distinctly uncommon, I and only a handful of cases has been reported.":" Of the patients with coronary murmurs described, the murmur has disappeareds-" or become difficult to hear' in those having subsequent coronary occlusion and myocardial infarction. Recent advances in coronary artery surgery utilizing saphenous vein grafts have permitted bypass of a localized stenosis, thereby relieving cardiac pain'': and improving ventricular performance.v-!? This paper describes the disappearance of a coronary artery diastolic murmur following aortocoronary bypass surgery. °From the Division of Cardiology, Department of Medicine, University of Oregon Medical School, Portland. Supported by Program Project Grant HL 06336 and Graduate Training Grant HL 05791 of the National Heart and Lung Institute. Reprint requests: Dr. Kassebaum, University of Oregon Medical School, 3181 SW Sam Jackson Park Road, Portland, Oregon 97201

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FIGURE 1. Phonocardiogram, indirect carotid pressure pulse and electrocardiogram (A) before aortocoronary bypass, (B) immediately after bypass, (C) following myocardial infarction two months later, and (D) ten months following surgery. In (A) an early systolic click (SC) is present, as well as systolic (SM) and diastolic (DM) murmurs, In (B) the systolic click and diastolic murmur have disappeared. In (C) the systolic click and diastolic murmur have reappeared. In ( D) the systolic click occurs a little later than previously and the diastolic murmur has disappeared.

CHEST, VOL. 63, NO.3, MARCH, 1973