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Rosai-Dorfman Disease : A Case Report With Review of L i t e ra tu re

GULL MOHD BHAT, SURENDER KUMAR, ATUL SHARMA

AB ST RA CTSinus his t iocytosis with massivelymphadenopathy (Rosai Dorfman disease)is an uncommon benign condition, oftenconfused with lymphoma. Patients usuallyhave massive enlargement of cervical lymphnodes. Clinical course is variable. Wedescribe here one such young male patientwith tonsi l lar enlargement . Pert inentliterature is reviewed.

INTRODUCTIONCastlemans disease, dermopathic lympha-denitis, mucocutaneous lymph node syndrome

(Kawasakis disease), histiocytic necrotisinglymphadenopathy (kikuchis disease), vascular t ransformation of lymph nodes andinflammatory pseudotumour of the lymph nodeare among the rare causes of lymph nodeenlargement. Sinus histiocytosis with massivelymphadenopathy (SHML) is an importantaddition to this list. An oncologist comes acrossa case every now and then with a cl inicalpicture hard to tell from malignant lymphomas.Histopathology usually comes as a surprise. Atthe same time it is a relief to the physician andthe patient, as this disease has essentially a

benign course.Case Report: -MSA, a 10 years young boy, resident of Bihar wasapparently alright about 3-4 months when henoticed a right neck mass, insidious in onsetand slowly increasing in size. Similar painlessswellings appeared on the other side of the neck,

Dept. of Medical Oncology, IRCHAll India Institute of Medical Sciences, New Delhi.Correspondence to : GULL MOHD BHATEmail : drgull8125@yahoo.com

both maxilla and epitroclear areas. The upper neck swellings progressed to attain the size of a

walnut. There was accom-panying history of intermittent low grade fever, lasting for a coupleof days unaccompanied by chills but usuallyassociated with cough and blood t ingedexpectorat ion. He had a s ingle episode of epistaxis; (on fol low up visi t had repeatepisodes of nasal bleed) and had beentransfused a unit of blood during this period.There has been no accompanying history of weight loss, night sweats or pruritis. His past,personal, and family history has been normal.Clinical examination showed that his weight

was 25 kgs, height 129 cms and had finger clubbing, bilateral tonsillar hypertrophy, a bullneck with multiple, generalized, non-tender,f i rm, and matted ( in cervical region)lymphadenopathy. In other areas lymph nodesvaried from 1 to 2 cms in size and (during followup developed lymph node which increased insize in armpits). His abdominal examinationrevealed moderate non-tender and soft / firmhepatosplenomegaly (5 & 2 cms below costalmargins) with no free f luid. Chest ,cardiovascular and central nervous system

examination was unremarkable.Investigations showed a hemoglobin of 8.2

WBC - 8500/cmm, defferential - N 63, E7, L30,platelets 180000/cmm. Peripheral blood filmshowed microcytic, hypochromic red bloodcel ls . ESR 58 mm/hr. Biochemical profi lerevealed normal renal and liver functions, withserum alkaline phosphates 225 units and LDH-64 units/ dl. Chest x-ray showed bilateral Hilar and Paratracheal lymphadenopathy.Chestimaging studies revealed mediast inallymphadenopathy. USG scan of the abdomen

Case Report-III

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revealed many peripancreatic and periportallymphadenopathy. FNAC of left cervical nodeshowed sheets of sinus histiocytes, some of which had evidence of emperopolesis and therewas presence of histiocytes and necrosis on theright side.

Lymph node biopsy showed markedlydilated sinuses containing sheets of histiocyteswith eosinophilic cytoplasm and small nuclei;some of the histiocytes show small lymphocytesin cytoplasm (emperipolesis). Scanty lymphoidt issue is seen in between. There was noevidence of malignancy or granulomas.Thesefeatures were consistent with a diagnosis of s inus his t iocytosis with massive lymphade-nopathy (Rosai-Dorfman Disease) (Fig. 1.A & B).

DISCUSSION:

This condition was first described by Robb-

Smith in 1947 in children and was termed asgiant cell sinus reticulosis . Sinus histiocytosiswith massive lymphadenopathy (SHML) hasbeen recognized as a distinct clinicopathologicalentity, though first given this name by Rosai andDorfman in 1969,1972. It has morphologicalfeatures of greatly exaggerated reactive process.There is usually no demonstrable etiologicalagent. The lymph nodes may attain enormoussize and may remain persistently enlarged for aprolonged period. The disease is not confined toa particular race or geographical area, although

Af rican Negroes are more commonly involvedthan whites. Characterized by massive, painlessand generally bilateral cervical lymph nodeenlargement with relatively minor involvementof other groups of nodes in most instances.

About 25% of cases have involvement of extranodal sites, especially the upper respiratorytract , sal ivary glands, orbi t and test is andlesions at other sites and skin may appear beforethe appearance of lymph nodes. 1 Patients withextensive lung and liver involvement may nothave as innocent course. 2 There may be osseousinvolvement in pediatric age group. Other raresites included solitary involvement of talus,triquetrum,sclera, conjunctiva, thymus, pleura,nasopharynx, genitourinary and central nervoussystem. These patients usually have moderatepyrexia with mild anemia, neutrophilia, a raisedESR and polyclonal gammapathy. Autoimmunehemolysis can also occur. Suggestion has beenmade that it may be an unusual reaction to aklebsiella infection. 3 Association to subsequentmalignancies is also described. 4 An infantpresenting with hepatosplenomegaly,bicytopenia considered as congenital form of

Rosai dorfman desease has been described.5

Thediagnosis is usually made on characteristichistopathological and cytological features.HUMARA assay shows the disease to bemonclonal.The cells have histochemical andphenotypic features of macrophages.The cellcontain large amount of esterases and acidphosphates and show positivity for CD 68 andalpha-1 antitrypsin.They also exhibit some of the phenotypic features of dendritic cells suchas S100, cathepsin E, fascin and at times CD 1a. 6,7

The natural history is that of a regression and

Fig 1. a Low power view (lymph node)showing massive distension of the sinuses

by the histocytic infilterate.

Bone marrow exami-nation revealed no evidenceof neoplastc infiltration, granulomas or parasiticinfections. Patient continued to be symptomaticand had high ESR. Lymph node aspiration wasrepeated. It reconfirmed the diagnosis. Repeatbone marrow aspirat ion/biopsy was unrem-arkable. The chi ld was given a course of ant ibiot ics and planned for br ief low dosecourses of steroids.

Fig 1. b High power view showinglymphophagocytosis (omperipolesis) by

the sinus histiocytes.

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resurgence followed eventually by completeresolution. No form of therapy has been found

to be of any benefit. In the Rosai and Dorfmanseries of 34 pat ients , one died of renalamyloidosis, another after cytotoxic therapy.The latter form of treatment is contraindicatedin this disease. Surgical option may be reservedfor compressive symptoms. 8, 9 Course in our patient was more protracted / progressive withno abatement of lymph node masses as withabout 6 months follow up; rather additionallymph node enlargement appeared in both axillnot iced in recent fol low up. Dominantnasopharyngeal involvement probably causedrecurrent epistaxis.

REFERENCES :

1. F o uc a r E l io t a nd R os ai J ua n an d Do r fm a n R F: S i nu shist iocytosis and massive lymphadeno-pathy.Ear,nose,throat manifestat ions. , Arch Otolaryngol. ,1978,104:687-693.

2. Foulard E, Rosa J, Dorman RF. Sinus histocytosis withmassive lymphadenopathy (Rosia- Dorfman disease):Review of the entity: Semin Diagn Patho 1990;7:19.

3. L a m pe r t F. a nd L en n e rt K : S i n us h is t i oc y to s i s w i thmassive lymphadenopathy. Fifeteen new cases. Cancer,1976,37:783-789.

4. R at z in g er J , Z ie g le r B eh o ld i ng Wet a l . Si n ushist iocytosis with massive lymphadenopathy. Arch.2003;443(6):797-800.

5. I w a bu c h i H , K ak i h ar a T, Ta n a ka A e t al . C on g e ni t al Rosai- Dorfman disease without lymphadenopathy.Pediatr Pathol Mol MED 2003 Sep-Oct;22(5) :399-403

6. E i s en RN , B u c kl e y P J , R o sa i J: I mm u n op h e no t y pi c characterization of sinus hist iocytosis with massivelymphadenopathy (Rosai- Dorfman disease) : SeminDiagn Patho 1990;7:74.

7. Jaffe R,Vaughan De.,Langhoff E:Fascin and differential

diagnosis of childhood histiocytic lesions. Pediatrol &Dev Pathol 1998;1:216.

8. D o ds o n K M , P o we r s C N , R e it e r E R , R o sa i D o r fm a ndisease presenting as synchronous nasal and intra-cranial mass Am J Otolaryngol 2003Nov-Dec;24(6),USA.

9. R a ve e n th i r an V, D ha n a la k a sh m i M , Ha y a va d a naRao,Vushwanath Rosai-Dorfman disease:report of a 3-year-old girl with critical review of treatment options.Eur J Pediatr Surg. Oct; 2003;13(5):350-4.