Eosinophilic granuloma of the rib

Eosinophilic A REVIEW COL. S. W.

OF THE FRENCH,

III,

Granuloma

RECENT M.C.

ENGLISH

LITERATURE,

Diplomate, American

From tbe Tboracic Surgery Section, Department of Surgery, Walter Reed Army Hospital, Walter Reed Army Medical Center, Wasbington, D. C.

the past eosinophiIic granuIoma of the rib as an uncommon Iesion.12 The author, in a recent report,g described two such cases seen within a year. The case reported in this articIe is the third seen in four years. This experience, as we11as the increasing number of reports in the Iiterature, suggests that this type of Iesion either is becoming more frequent or is being recognized more often. In 1933 Fraser8 adequateIy described this bone Iesion and gave it the name Iipoid granuIomatosis. Farber6 in rg4r suggested that the Iesion was cIoseIy associated with those of SchtiIIer-Christian disease (IipogranuIomatosis). Lichtenstein and Jaffe in rg40i3 gave the disease its present name of eosinophiIic granuIoma and in ig4411 suggested that it represented an inIIammatory reaction caused by an unknown infectious agent. Others suggest that the condition is cIoseIy associated with Letterer-Siwe disease (non-Iipoid histiocytosis). The consensus appears to be that a11 three conditions, SchtiIIer-Christian disease, Letterer-Siwe disease and eosinophilic granuIoma are variants of the same disease.3016

I has been described N

CLINICAL

AND

LABORATORY

FINDINGS

It was formerIy thought that eosinophilic granuIoma was primariIy a disease Iimited to chiIdren and young aduIts, and that its occurrence was extremeIy rare after the age of twenty-five years. Recent reports, however , 3,7,g*10,15,16*1g indicate that this condition occurs commonIy in the fourth, fifth and even the sixth decades of Iife. It is found most commonIy in ribs and other ffat bones such as those in the skuI1, scapuIa, mandibIe and peIvis. It has aIso been discovered in the femur and vertebral bodies but has never been reported in the bones of the hand and feet. Thedisease may occur as an isoIated Iesion or as muItipIe

of the Rib WITH

A CASE

REPORT

Board of Surgery, San Francisco,

Calijornia

Iesions, affecting bones’* and other organs such as the Iungs and diaphragm.‘~” It affects maIes more often than femaIes.3 A hereditofamiIia1 tendency, however, has not been demonstrated.12 History of trauma is uncommon. The symptomatoIogy usuaIIy consists of Iow grade pain over the affected area and may be associated with a miId febriIe reaction.Ssl* There is usuaIIy sweIIing and tenderness over the affected area. Less common systemic manifestations incIude weight Ioss, anorexia and maIaise. If the Iesion is observed over a considerabIe period of time, there is usuaIIy a rather rapid progression on radioIogic examination.2 X-ray reveaIs a fusiform sweIIing over the affected area with osteoIysis of the meduIIary portion of the bone. The cortex may or may not be affected, but in any case it is usuaIIy very thin. PathoIogic fractures may occur. Spontaneous healing4 and cures by x-ray therapy and simpIe curettage have been reported. EosinophiIia may range from o to I I per cent.3*5,14 It shouId be emphasized, however, that histoIogic examination is necessary in order to estabIish a definite diagnosis, and excision is the treatment of choice. Eosinophilic granuIoma must be differentiated from metastatic carcinoma, simpIe bone cyst, Ewing’s sarcoma, reticuIum ceI1 sarcoma, myeIoma, Iymphoma, osteochondroma, as we11 as fibrous dyspIasia of bone, osteomyeIitis and osteitis fibrosa cystica. ObviousIy, histoIogic examination is mandatory in order to ruIe out the aforementioned conditions. CASE

REPORT

A twenty-nine year oId white maIe physician stated that in May, 1953, whiIe Iifting a dresser, he deveIoped back pain about the IeveI of the fifth thoracic vertebra. The pain persisted for severa days but x-rays reveaIed no pathoIogic condition. In June the patient noticed the onset of intermittent right anterior chest pain which 627

American

Journal

of Surgery,

Volume

88, October,

,954

Eosinophilic

GranuIoma

FIG. I. Roentgenogram demonstrating the osteolytic Iesion of the anterior portion of the right sixth rib showing meduIIary and cortica1 invoIvement.

he beIieved to be related to the previous injury, but x-rays were again negative. On JuIy 18th, because of severe pain, further x-ray studies were made. These showed a Iesion in the anterior portion of the right sixth rib. AdditionaI flms taken on August 22nd showed a definite increase in the size of the destructive Iesion in the rib. (Fig. I.) Except for pain there were no other compIaints. On physica examination the patient was we11 deveIoped, we11 nourished and quite apprehensive but did not appear acuteIy iI1. His blood pressure was 126/70 and his puIse 80. There was a tender area measuring I by 2 cm. over the site of the Iesion in the sixth rib anteriorIy, but no tumor was paIpabIe. A compIete brood count and urinaIysis were within norma limits. There was no eosinophiIia. Bence-Jones protein test was negative. Excretory urogram was normaI. Chest x-ray reveaIed the Iesion in the right anterior sixth rib to have increased in size. It appeared to be a destructive Iesion which invoIved the cortex of the bone. The foIIowing report by the radioIogist iIIustrates the uncertainty of the radiologic diagnosis: “On the anterior portion of the sixth right 628

of Rib

rib is an irreguIar osteoIytic Iesion confined more to the superior portion of the rib than the inferior and containing areas of radioIucency with some areas of scIerosis. There is destruction of the superior cortex. No periostea1 reaction and no masses were seen. A metastatic Iesion is to receive first consideration. A primary malignant bone Iesion of the non-ossifying type may aIso be considered. An eosinophiIic granuIoma might give this appearance, aIthough benign Iesions are not considered because of destruction of the cortex.” A skeIeta1 survey on admission reveaIed no other Iesions. On August 28th, using gas-oxygen-ether endotrachea1 anesthesia, an 8 cm. portion of the invoIved sixth rib, aIong with the intercostal muscIe bundIe and pIeura on either side of the rib, was excised en bloc. The patient withstood the procedure well’and his convaIescence was uneventfu1. A smear of the Iesion taken after its remova reveaIed many paIe-staining eosinophiIs. Stained paraffin section examination was reported as foIIows: “Sections taken through the Iesion revea1 a process which is broken through the outer cortica1 area at one point and has projected IateraIIy and which descends into the subperiostea1 region. The mass of tissue is composed predominantIy of an interIacing pattern of fibroblasts which in some instances is quite dense and in others shows some myxomatous and mutinous stroma. This tissue is quite vascuIar. There is a marked infiItration, somewhat irreguIar, of eosinophiIs. There are aIso present some Iymphocytes and pIasma ceIIs. The predominating ceIIs, however, other than fibrobIasts, are histiocytes and macrophages. SmaII foci of necrosis can be seen and about these areas are occasiona muItinucIeated foreign-body type giant ceIIs. This process has diffuseIy infiItrated the bony trabecuIae and areas of osteoIytic activity are noted. Where this process is subperiostea1 in Iocation there is in addition foci of new bone formation.” SUMMARY

A review of the recent EngIish Iiterature on eosinophiIic granuIoma of the rib is presented. Excision en bloc and histoIogic examination are necessary to differentiate this lesion from other diseases having a simiIar radioIogic appearance and clinica picture.

Eosinophdic

Grandoma

REFERENCES

I. ACKERMAN, A. .I. Eosinophilic granuloma of bones associated with involvement of Iungs and diaphragm. Am. J. Roentgenol., 58: 733, 1947. 2. BAKER, W. .I., HOUGHTON, J. D., WISSING, E. and BETTS, R. H. Eosinophilic grantdoma: report of case with x-ray evidence of rapid progression. New England J. Med., 238: 626-629, 1948. 3. CARTER, S. B. Eosinophilic granuloma of rib. J. M. A. Georgia, 41: 17-18, 1952. 4. COLEY, B. L. Neoplasms of Bone and Related Conditions, pp. 611-616. New York, 1949. PauI B. Hoeber. 5. DUNDON, C. C., WILLIAMS, H. A. and LAIPPLY, T. C. Eosinophilic granutoma of bone. Radiology, 47: 433-444, 1946. 6. FARBER. S. Nature of solitary or eosinophilic granuIoma. Am. J. Patb., 17: 625-629, 194;. 7. Fox, R. T. and CARSWELL, J., JR. Solitary eosinophiIic granuloma of rib. Am. J. Surg., 82: 4024.04, ‘951. 8. FRASER, J. Lipoid granulomatosis of bones. Brit. J. Surg., 22: 800, 1935. g. FRENCH, S. W., III. Eosinophilic granuIoma of the rib. U. S. ArmedForces M. J., 2: 1681-1684, 1951. IO, GRANT, A. R., HOUSE, R. K. and CRANDELL, W. B. EosinophiIic granuloma of rib. New England J. Med., 240: 541-543,

1949.

of Rib

II. JAFFE, H. L. and LICHTENSTEIN, L. EosinophiIic granuIoma of bone; condition affecting one, severa1 or many bones, but apparently Iimited to skeIeton, and representing miIdest clinica expression of peculiar inffammatory histiocytosis underIying Letterer-Siwe disease and aIs SchiiIIer-Christian disease. Arch. Patb., 37: 99-r 18, 1944. 12. KIPP, H. A. and FISHER, E. R. Eosinophilic granuIoma of rib with a case report. J. Tboracic Surg., 21: 24-29, 1951. 13. LICHTENSTEIN, L. and JAFFE, H. L. Eosinophilic granuioma of bone. Am. J. Path., 16: 595604, 1940. 14. MAURER, E. and DESTAFFANO, G. A. Eosinophilic granuIoma of rib. J. Tboracic Surg., 17: 35~356,

I 948.

15. RAMSAY, A. G. EosinophiIic granuloma of rib. Treat. Serv. Bull., 7: 237-240, 1952. 16. SHAFIROFF, B. G. and SCHEMAN,L. SoIitary eosinophilic granuIoma of rib. Ann. Surg., 125: 5 IO512, '947. I7. STREETE, B. G. Persona1 communication. 18. THURM, A. S. EosinophiIic granuloma of bone, report of case with multiple lesions. Bull. Hosp. Joint Dis., 3: 9-16, 1942. rg. WEIR, D. R. Eosinophilic granuIoma of rib. Ann. Int. Med., 35: 233-236, rg5 1.