Orbital Involvement in 'Sinus' Histiocytosis A David S.

\s=b\ Sinus histiocytosis is a newly recognized benign disease affecting mainly children and young adults and usually having a protracted clinical course that is relatively unaffected by therapy. This paper describes four additional patients who had orbital involvement initially and reviews the salient clinical and histopathologic features of this entity. The outstanding clinical feature is cervical lymphadenopathy. Associated findings include low-grade fever, anemia, leukocytosis, and elevated lgG levels. A small percentage of patients develop proptosis with palpable orbital tumors. Such pa-

histiocytosis with massive lymphadenopathy has become an established clinicopathologic entity primarily as a result of the publica-

Sinus

tions of Rosai and Dorfman.'-2 Although several case reports had been published previously,'" the four cases reported by Rosai and Dorfman in 1969' and the 30 additional cases studied by the same authors in 1972clearly delineated the clinical and pathologic features of this entity. The disease is clinically characterized by massive painless enlargement of lymph nodes, particularly those in the cervical area (proconsular neck). for publication March 3, 1977. From the Registry of Ophthalmic Pathology, Armed Forces Institute of Pathology, and the Departments of Ophthalmology, Georgetown University Medical Center (Drs Font and Rao) and Children's Hospital National Medical Center

Accepted

(Dr Friendly), Washington.

The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Reprint requests to Children's Hospital National Medical Center, 2125 13th St, NW, Washington, DC 20009 (Dr Friendly).

Report of

Friendly, MD; Ramon

L.

Four Cases Font, MD; Narsing A. Rao, MD

tissues, including the palatine tonsil, nasal mucosa, orbit, eyelid, skin, and testicle.2 Three of the 34 patients described by Rosai and

appreciable lymphadenopathy. Progressive proptosis may

affect other

eye.

Dorfman2 had involvement of the eyelid or orbit, one of whom (case 23) had been reported earlier by Destombes.7 Three additional patients with orbital involvement have been

tients may not have

lead to exposure keratitis, corneal ulceration, endophthalmitis, and loss of the the lymph nodes and orbital mass show a proliferation of large histiocytes intermixed with a variable proportion of lymphocytes and plasma cells. Lymphocytes and occasionally other cells derived from the hematopoietic system are commonly seen within the cytoplasm of the histiocytes.

Histopathologically,

(Arch Ophthalmol 95:2006-2011, 1977)

Cervical adenopathy was present in 33 of the 34 patients described by Rosai and Dorfman.2 These authors found slight enlargement of axillary and inguinal lymph nodes in 38% and 44% of their cases, respectively, and 42% had roentgenographic evidence of hilar and/or mediastinal lymphadenopathy. The lymphadenopathy develops gradually, may persist for months or years, and eventually regresses. Other associated findings include low-grade fever, leukocytosis with neutrophilia, elevated ESR, and hypergammaglobulinemia. Most affected persons are children who appear to be otherwise healthy. Although the disease probably occurs more frequently in black patients, the number of white patients slightly exceeds the number of black" in 62 reported cases.12" The disease is not believed to be genetically determined and occurs equally in both sexs. The cause is obscure, and there is no recognized effective treatment. Although involvement of lymph nodes in sinus histiocytosis is characteristic, the disease frequently may

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reported."

"'7

The purposes of this paper are (1) to describe four additional patients with sinus histiocytosis who presented initially with orbital involvement and (2) to review the salient clinical and histopathologic features of this entity. REPORT OF CASES

9-year-old Nigerian boy had cervical lymphadenopathy and a left orbital mass. Ipsilateral corneal ulcération and Case l.-A

secondary endophthalmitis

developed,

which necessitated enucleation of the ocular contents. An x-ray film of the right ulna revealed a radiolucent bony defect. A biopsy of this lesion suggested the possibility of histiocytosis X. The patient was treated with vinblastine sulfate and prednisolone. A subsequent left cervical lymph node biopsy evaluated in Nigeria was interpreted as not being consistent with malignant lymphoma. Neither slides nor wet tissue from either biopsy specimen were available for review at the Armed Forces Institute of Pathology (AFIP). A few months later, the orbital tumor allegedly recurred, and 3 gm of "yellowish hard nodular tumor tissue" was excised. Histopathologic examination in Nigeria led to the diagnosis of orbital pseudotumor. Recurring moderate cervical lymphadenopathy prompted additional lymph node biopsies; these tissues were interpreted as nonspecific reactive lymphadenopathy. Sections from the lymph nodes and orbital tumor were submitted to the AFIP for histopathologic examination. No additional

4. Computed tomographic with contrast enhancement showing bilateral orbital masses prior to biopsy

Fig 2.—Case scan

(AFIP Neg 76-1355).

Fig 1 .—Case 4 at age 4 years showing bilateral symmetric proptosis with palpable orbital masses superiorly (AFIP Neg 76-1356). Table 1.—Clinical Features of Four Patients With Sinus Histiocytosis Duration of

Age at Diagnosis,

Case

Race

1

yr 9

Sex M

Nigerian

2

10

F

Nigerian

Proptosis

Orbital Location Left

Prior to Orbital Biopsy NS*

black

2

3

F

M

"NS,

black American black American black

Left superior and lateral Left medial

Right and left superior

Moderate cervical NS

3

mo

Moderate cervical

8

mo

Slight

Fig 3.—Case 4. After radiation therapy, the left eye developed corneal ulcer interiorly (AFIP Neg 77-1045).

cervical

not stated.

clinical details or follow-up information

are

available.

Cask 2.-A 10-year-old Nigerian girl was first examined in an eye hospital in Nigeria because of "progressive swelling of the left eye for five years." Visual acuities were 20/25 in the right eye (OD) and 20/30 in the left eye (OS). The right eye and orbit were entirely normal. X-ray films of the orbits revealed no bony abnormalities. On July 25,1975, the left orbit was surgically explored, and a "very firm, lobular yellow fatty tumor occupying all of the upper and temporal quadrants of the orbit" was found. The entire mass was forwarded to the AFIP for histopathologic evaluation. The clinical diagnosis was librolipoma or mixed tumor of the lacrimal gland. Unfortunately, the patient was lost

to

5 yr

Adenopathy

follow-up.

2-year-old American black three-month history of progressive enlargement of a subconjunctival mass in the medial canthal region. The Case 3.—A

girl had

a

globe

was

displaced laterally.

Moderate

submandibular and bilateral anterior and

posterior cervical lymphadenopHthy

was

noted. A roentgenogram of the chest was within normal limits. Surgical excision of the mass was performed in October 1975. The tumor was found overlying the left medial reclus muscle and extended approximately IV2 cm posteriorly. Five months postoperatively there was no evidence of recurrence. The biopsy specimen was sent to the AFIP for histopathologic examination. The patient's current status is unknown. Case 4.-This patient was submitted by the Department of Ophthalmology of Children's Hospital National Medical Center, Washington. This patient was under the care of one of us (D.F.) and will be described in detail. A 20-month-old black boy was hospitalized on Dec 18,1971, for an acute episode of polyarthritis and bilateral otitis media. At that time a generalized urticarial eruption

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Fig 4.—Case 4. Left fundus showing blurring of disc margins and moderate dilatation of retinal veins (AFIP Neg 76-1353).

nonpainful swelling of the joints of the knees, wrists, and hands were observed. Slightly enlarged posterior cervical and submandibular lymph nodes were noted. No ocular or orbital abnormalities or hepaand

tosplenomegaly were

recorded. revealed a hemogloml and a WBC of 16,400/cu mm with 60 segmented neutrophils, 36 mature lymphocytes, three eosinophils, and one basophil. The ESR was 17

Laboratory studies bin of 11.6 gm/100

Fig 5.—Case 1. defined lobules

Low-power view of orbital mass disclosing wellcomposed of histiocytes and focal collections of lymphocytes separated by broad fibrous septa. The overall picture resembles sclerosing inflammatory pseudotumor (hematoxylin-eosin, x 25; AFIP Neg 76-3319). mm/hr corrected. A chest x-ray film showed no evidence of mediastinal enlargement. A sickle cell preparation, urinalysis, and tilers for rubella, antistreptolysin, and latex fixation were within normal limits. Joint swelling and fever subsided on orally administered penicillin

therapy.

Slowly progressive, painless, symmetric proptosis became apparent in June 1974. Intermittent fever and a 0.9-kg weight loss occurred in the four months prior to the second hospitalization on Feb 26, 1975. Abnormal physical findings at this time

included a temperature of 37.7 C and bilateral proptosis (Hertel measurements, 24 mm bilaterally; base, 90 mm). Bilateral,

lobulated, firm, rubbery masses measuring approximately 12 X 8 mm were palpable through both upper lids. The globes were displaced forward and downward (Fig 1),

and marked resistance to retrodisplacement was noted. Except for slight limitation of upward gaze, rotations and ocular alignment were unimpaired. No diplopia was present. Visual acuity was 20/30 bilaterally; applanation tensions were 12 mm Hg bilaterally. The pupils reacted normally to light, and the fundí appeared unremarkable. Slight cervical lymphadenopathy, thought to be clinically insignificant, was recorded. The hemogram revealed a hemoglobin level of 11.5 gm/100 ml and a WBC of 9,400/cu mm with 26 segmented neutrophils, 73 mature lymphocytes, and one monocyte. Chest, skull, and orbital x-ray films, including orbital tomograms, were normal. Findings from thyroid-function studies, including a radioiodineuptakc and a liothyronine sodium-suppres-

Fig 6.—Case 2. Orbital mass showing large histiocytes with pale vesicular nuclei containing mature lymphocytes in their cytoplasm. Lymphoplasmacytic infiltrate surrounds histiocytes (hematoxylin-eosin, x350; AFIP Neg 76-3320).

sion test, were within normal limits. The results of other studies, including urinalysis, routine chemical analyses of the blood, protein and lipoprotein electrophoreses, 24hour urinary vanillylmandelic acid excretion, and VDRL for syphilis were normal. Immunoglohulin electrophoresis revealed IgA, 290 mg/100 ml; IgG, 1,850 mg/100 ml; and IgM, 235 mg/100 ml. Contact B-scan ultrasonography with the Bronson-Turner unit showed circumscribed sonolucent superior orbital and retrobulbar masses. Computed tomography demonstrated bilateral, discrete orbital tumors (Fig 2), which were thought to be slightly vascularized. On March 7, 1975, a biopsy of the right orbital mass was performed. A discrete,

nonencapsulated, relatively avascular, tongue-like projection of tissue measuring 1.0 x 5 x 1.0 cm was excised through an upper lid incision. Histopathologic exami-

nation of this material at the AFIP established the diagnosis of sinus histiocytosis. The patient was discharged from the hospital shortly after the surgical procedure. On April 4, 1975, a three-month trial of prednisonc therapy was initiated, with no observable effect on the proptosis. In late September and early October 1975 a course of radiation therapy to the left orbit was given (a total dose of 1,200 rads). The patient was seen two weeks later and had increased proptosis of the left eye and an infected left corneal ulcer (Fig 3). A 3 x 1-mm infiltrate at the level of Bowman's membrane with overlying corneal epithelial necrosis was present interiorly. Flare and cells were noted in the

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anterior

chamber, but

no

hypopyon

was

present.

Firm orbital masses, more prominent in the left orbit, could be palpated superiorly and anteriorly, and the left globe was 1 to 2 mm more proptotic by Hertel measurements. Mild cervical lymphadenopathy was again noted. Acinetobacter calcoaceticus (Mima) organisms sensitive to neomycin sulfate, oxacillin sodium, and gentamicin sulfate were isolated from scrapings of the left corneal ulcer. The corneal ulcer subsided when the patient was administered topically an ointment containing polymyxin B sulfate, neomycin sulfate, and bacitracin zinc (Neosporin) and systemically ampieillin sodium therapy. Early in November, ophthalmoscope examination of the left eye revealed 2 to 3 diopters of elevation of the optic disc and dilated, tortuous retinal veins (Fig 4). No hemorrhages or exúdales were apparent. Visual acuities at this time were 20/30 OD and 20/40 OS, and Hertel measurements wore 25 mm OD and 20 mm OS. An Epstein-Barr virus serum antibody titer was 128. When the patient was next seen, on Feb 5,1976, no definite evidence of papilledema was noted, although the right disc margins were somewhat more distinct than the left. Hertel measurements were 28 mm OD and 31 mm OS. Diffuse punctate keratitis was

present bilaterally.

The increasing proptosis and failure of medical treatment prompted transcranial orbital decompression and partial resection of tumor tissue on Feb 28. Bilateral tarsorrhaphies were also performed. When the orbits were unroofed, yellowish, rub-

Fig 7.—Case 3. Left, Orbital mass disclosing bizarre, binucleated and multinucleated histiocytic cells and scattered mature lymphocytes (hematoxylin-eosin, X225; AFIP Neg 76-3312). Right, Highpower view of adjacent fields showing several atypical histiocytes with hyperchromatic nuclei containing prominent single nucleolus (hematoxylin-eosin, X485; AFIP Neg 76-3312).

Fig 8.—Case 2. Infiltration of lacrimal gland by orbital mass composed of admixture of lymphocytes, plasma cells, and large histiocytlc cells (hematoxylin-eosin, X110; AFIP Neg 76-3322).

bery

orbital tumor tissue immediately welled up. As far as could be ascertained, tumor was not present within either muscle cone. Several grams of tissue were removed from each orbit. The orbital defects were not closed. One month postoperatively the visual acuities were normal, and no corneal staining was present. In March 1976 the Hertel measurements were 24 mm OD and 26 mm OS. Residual tumor tissue was still palpable through both upper eyelids. The major clinical features of these four cases are summarized in Table 1.

Pathologic Findings Grossly, the orbital mass in

all cases lobulated firm tumor that was sharply delineated from surrounding tissues but was not encapsulated. The cut surface displayed well-circumscribed yellowish-white areas surrounded by fibrous

consisted of

septa.

a

Microscopically the orbital lesions were composed predominantly of lobules of vari-

able size and shape, surrounded by dense bands of connective tissue (Fig 5). These lobules were made up of a polymorphic infiltrate composed of nonneoplastic histiocytes admixed with large numbers of lymphocytes and plasma cells. Most of the histiocytes were large and had round to oval nuclei with a distinct nucleolus and a slightly vacuolated cytoplasm. A characteristic feature frequently observed was the phagocytosis of lymphocytes, erythrocytes, and plasma cells in varying numbers (Fig 6) in the cytoplasm of some of the large histiocytes. In some histiocytes the intra-

cytoplasmic lymphocytes were arranged around the periphery, forming a nuclear crown. Atypia and mitotic figures were usually absent. Case 3, however, showed atypical histiocytes with large pleomorphic nuclei and prominent nucleoli (Fig 7). Some of these

histiocytes

fused to form small,

multinucleated giant cells.

Lymphocytes frequently constituted a prominent component of the cellular infiltrate. Most of the lymphocytes were

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admixed in variable proportions with the large histiocytes. Occasionally the lympho-

cytes formed ill-defined follicles. Moderate

numbers of plasma cells and Russell bodies were also present. In one case this polymorphic cellular infiltrate extended into the parenchyma of the lacrimal gland

(Fig 8).

The cervical lymph node from case 1 showed histopathologic findings similar to those observed in the orbital lesions. The lymph node, which exhibited less fibrosis, typically displayed dilatation of the subcapsular and medullary sinuses containing large numbers of histiocytes, plasma cells, and Russell bodies (Fig 9). Occasional residual lymphoid follicles were observed. COMMENT

Sinus histiocytosis may present with primary orbital involvement. Indeed, the lymphadenopathy may be so insignificant as to be clinically overlooked. The proportion of patients with sinus histiocytosis who develop

9.—Case 1. Left, Lymph node showing dilatation of medullary sinuses filled with large histiocytes. Prominent plasma cell infiltrate is present on the left. Lymphoid follicle is seen on the right (hematoxylin-eosin, x 200; AFIP Neg 76-3317). Right, High-power view of area adjacent to that shown at left displaying phagocytosis of lymphoid cells within cytoplasm of histiocyte (arrow) (hematoxylin-eosin, x 440; AFIP Neg 76-3316).

Fig

orbital involvement is not known. The incidence can be estimated, however, by noting that three of the 34 patients described by Rosai and Dorfman in 19722 had either eyelid or orbital involvement. Clinical features of the four patients with orbital involvement described here are summarized in Table 1. Five additional patients who had orbital involvement are summarized in Table 2. Only two of the nine patients with orbital involvement had what might be termed massive lymphadenopathy, and in two patients the lymphadenopathy was described as moderate. In four patients it was slight and in one patient was not quantitated. The fact that some patients with orbital tumor may not have massive lymphadenopathy indicates that the term used by Rosai and Dorfman,'-2 "sinus histiocytosis with massive lymphadenopathy," is not entirely appropriate. Another difficulty with this designation concerns the histogenic concept advanced by Rosai and Dorfman'-2 of a remodeling of lymph node architecture as the basic structural pathologic alteration. The remarkable similarity of the histopathologic features in extranodal tissues, such as the skin, testicle, nose, orbits, and palatine tonsil, to those observed in the affected lymph nodes implies that the above concept is inadequate in itself to account for all the

Table 2.—Clinical Features of Five Previously Described Patients With Sinus Histiocytosis With Orbital Involvement Duration of

Age at Diagnosis, Case 1

yr_Sex

20

M

Race White

20

F

Martinique black Pakistani

11

12

M

M

"The lymph nodes

were

Right superior

12 yr

and inferior Right and left superior and inferior

3 yr

African black

Right

American black

Right and left superior

examined

Proptosis

Orbital Location Left lateral

and

Prior to Orbital Biopsy NS

Adenopathy Marked hilar and mediastinal Marked cervical *

Slight cervical and

12

mo

left

axillary*' Slight mandibular

Several weeks

Slight

cervical and mandibular*

histopathologically.

sites of involvement, as suggested by Zimmerman.21 Furthermore, the term "sinus," which obviously refers to the sinuses of lymph nodes, is not applicable to extranodal tissues. The orbital mass is generally located in the peripheral surgical space rather than within the muscle cone. In most cases visual acuity remains good, and functionally severe involvement of extraocular muscles is unusual. Increasing proptosis, however, may lead to exposure keratitis, corneal ulcération, and endophthalmitis, necessitating enucleation of the

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as in case 1 of our series, as well in the case reported by Destombes et al.'" The cause of sinus histiocytosis is obscure. The elevated IgG levels found in most cases are consistent either with a primary disturbance in immune mechanisms or an infectious process. The former possibility was investigated in discordant identical twins. Transiently deficient responses to phytohemagglutinin stimulation and transiently decreased phagocytic function by the nitroblue tetrazolium test were found in both siblings.22 In

eye, as

two additional

cases

investigated by

techniques, however, other investigators" failed to find any evidence of an immune deficiency. Results of lymphocyte-function tests were normal also in a recently studied

the

same

patient." The possibility of a primary abnormality of host histiocytes unrelated to an impaired immune response was raised by the experimental work of Karpas and colleagues." Working with in vitro preparations, these investigators found that cultured histiocytes from an affected patient had the capacity to phagocytize buffy coat

cells from a normal person. No microorganisms have been morphologically identified in the affected tissues of patients with sinus histiocytosis. Indirect evidence of EpsteinBarr virus infection has been presented by Rosai and Dorfman2 and by Lober et al." Seven of 22 patients studied recently by Sanchez and coworkers21 failed to demonstrate an elevated Epstein-Barr virus titer, as did the patient studied by Lamport and Lennert." It is interesting in this respect that the single patient in the present series on whom the test was performed (case 4) had elevated Epstein-Barr virus antibody titers. The presence of elevated antibody

titers to Klebsiella antigens was described in a recent report." The authors emphasized the similarity of the histopathologic findings to those observed in rhinoscleroma caused by K rhinoscleromatis. The possibility that the disease is a chronic infection by Klebsiella organisms awaits investigation of additional patients. Potentially fatal diseases that might be considered clinically in differential diagnosis of children with orbital involvement include malignant lymphoma (especially Burkitt's tumor), granulocytic sarcoma, rhabdomyosarcoma, and metastatic neuroblastoma. These disorders can generally be distinguished on the basis of rapid clinical progression, x-ray findings, and appropriate laboratory studies. Histiocytosis X (which, despite the similarity of name, is completely unrelated to sinus histiocytosis) can lie distinguished by the characteristic clinical manifestations and/or roentgenographic findings of the three conditions it comprises. Orbital extension of retinoblastoma should be clearly suspected on the basis of the ophthalmoscopic findings. Sinus histiocytosis with orbital involvement may be misclassified histopathologically as a variant of inflammatory

pseudotumor or xanthogranulomatous

disorder. The microscopic findings of sinus histiocytosis are sufficiently distinctive to permit differentiation from the latter two entities. Codling et al have described a complete differential diagnosis." The effectiveness of any form of treatment is uncertain. Apparent improvement after radiation therapy" and steroid treatment must be judged with caution in view of the absence of controls and the self-limited nature of the disease. Case 4 in our series showed no improvement in the proptosis after treatment with radiation and systemically administered steroids. This investigation was supported in pari by Public Health Service training grant EY-00082 from the National Eye Institute. Roma Chandra, MI), Chief of Pathology, Children's Hospital National Medical Center, and Juan Rosai, Ml), advised in the clinical management of case 4. Ho also confirmed the histopathologic diagnoses of cases 3 and 4.1'aul Gerber, MD, of the National Institutes of Health performed the scrum antibody titer for case 4.

Nonproprietary Names and Trademarks of Drugs Ampicillin Boäiüm—Alpen-N, Awrill-S, Omnipen-N, Penbrilin-S, Pplycilliti-N, Principen/N. Gentamicin sulfate—Garamycin, Vinblastin sulfate— Vetban.

References 1. Rosai J, Dorfman RF: Sinus histiocytosis with massive lymphadenopathy. Arch Pathol

87:63-70,

1969. 2. Rosai J, Dorfman RF: Sinus histiocytosis with massive lymphadenopathy: A pseudolymphomatous benign tumor. Cancer 30:1174-1188, 1972. 3. Lennert K: Lymphknoten, cytologie und lymphadenitis, in Handbuch der Speziellen Pathologischen Anatomie und Histologie, vol 1, pt

3/A.

Berlin-Gottingen-Heidelberg, Springer,

1061, p 504.

4. Azouri FJ, Reed RJ: Histiocytosis: Report of Med 274:928-930, an unusual case. N Engl J

1966. 5. Vincent TN, Miercourt R: Histiocytosis of the eyelid. Penrose Cancer Hosp Bull 3:246-250, 1967. 6. Marie J, Bernard J, Nezelof C, et al: Adenop-

athies chroniques

histiocytaire et

42:2689-2698, 7.

proliferation reticulosurcharge lipidique. Ann Pediatr avec

1966. Destombes P: Ad\l=e'\nitesavec surcharge lipidique de l'enfant ou de l'adulte jeune, observ\l=e'\es aux Antilles et au Mali (quatre observations). Bull Soc Pathol Exot 58:1169-1175, 1965. 8. Dhermy P, Destombes P, Rousselie F: Localisation orbitaire de l'histiocytose macrophagique lymphocytaire (histiocytose sinusale de Rosai).

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histiocytosis

with massive

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Cancer 37:783\x=req-\

17. Pickering LK, Phelan E: Sinus histiocytosis. Pediatr 86:745-748, 1975. 18. Mehrotra D, Saxena H, Samuel KC, et al: Benign sinus histiocytosis with massive lymphadenopathy simulating Hodgkin's disease. Indian Cancer 12:224-227, 1975. 19. Chandrasekar S, Veliath AJ, Chaudhuri S, et al: Sinus histiocytosis with massive lymphadenopathy. Assoc Physicians India 23:252-528, 1975. 20. O'Grady RB: Sinus histiocytosis\p=n-\acase with orbital involvement. Read before the Georgiana Dvorak Theobald Society, Louisville, Ky, 1971. 21. Zimmerman LE: Inflammatory pseudotumors of the orbit. Read before the Verhoeff J

10. Destombes

P, Destombes M, Martin L:

Histiocytose lipidique ganglionnaire pseudo-

tumorale: Nouvelle observation chez une jeune Martiniquaise. Bull Soc Pathol Exot 65:481-488, 1972. 11.

Codling BW, Soni KC, Barry DR, et al: Histiocytosis presenting as swelling of orbit and eyelid. Br Ophthalmol 56:517-530, 1972. 12. Lennert K, Niedorf HR, Blumcke S: J

with massive hemophagocytic sinus histiocytosis. Virchow's Arch (Cell Pathol) 10:14-29, 1972. 13. Lober M, Rawlings W, Newell GR, et al: Sinus histiocytosis with massive lymphadenopathy: Report of a case associated with elevated EBV antibody titers. Cancer 32:421-425, 1973. 14. Karpas A, Arno J, Cawley J: Sinus histiocytosis with massive lymphadenopathy: Properties of cultured histiocytes. Eur J Cancer 9:729-732,

Lymphadenitis

1973. 15. Jim\l=e'\nezAV, Gonz\l=a'\lezJF, Mart\l=i'\nezAM, et al: Histiocitosis sinusal con linfoadenopat\l=i'\a masiva de Rosai y Dorfman. Patologia 6:263-272, 1973. 16. Sinclair-Smith CC, Kohn LB, Uys CJ: Sinus

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Afr Med

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J

J

Society meeting, Washington, DC, April 24, 1974. 22. Becroft Benign sinus

DMO, Dix MR, Gillman JC, et al: histiocytosis with massive lymphadenopathy: Transient immunological defects in

a

child with mediastinal involvement.

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J

Clin

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extranodal manifestations. Abstracts of papers read before the 66th annual meeting of the International Academy of Pathology, Toronto, March 16, 1977.

Orbital involvement in 'sinus' histiocytosis. A report of four cases.

Orbital Involvement in 'Sinus' Histiocytosis A David S. \s=b\ Sinus histiocytosis is a newly recognized benign disease affecting mainly children and ...
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