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 Table of Contents  
Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 45-47

Management of a patient with Rosai–Dorfman syndrome and large adrenal pheochromocytoma

1 Department of Medicine, Faculty of Medicine, School of Medicine, University of Sulaimani, Sulaymaniyah, Iraq
2 Department of Haematology, Hiwa Hospital, Sulaymaniyah; Department of Haematology, Kurdistan Board for Medical Specialties, Ministry of Higher Education and Scientific Research, Erbil, Iraq

Date of Submission06-Dec-2017
Date of Acceptance20-Jan-2018
Date of Web Publication13-Feb-2018

Correspondence Address:
Dr. Najmaddin Khoshnaw
Department of Haematology, Hiwa Hospital, PO. Box: 54 at Al-Sulaymaniyah Post Office, Sulaymaniyah, Kurdistan Region
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijh.ijh_45_17

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Sinus histiocytosis with massive lymphadenopathy that known as Rosai Dorfman syndrome (RDS)is a rare histiocytic disease which includes benign lymph nodes (LN) enlargement with extra-lymphatic soft tissue involvement. The concomitant presentation of RDS with an adrenal tumor is rarely encountered among our patients. Here in we describe a middle-aged man, presented with a chronic history of repeated LN enlargement. The nodes had mild local discomfort and tenderness with associated fever. LN biopsy showed sinus histiocytosis with lymphophagocytosis characterized as emperipolesis and diagnosis of RDS was decided. After one year, the patient presented with high blood pressure, raised blood sugar, obesity, and abdominal pain. Computerized tomography showed a large adrenal mass, and the patient was treated urgently for his malignant blood pressure followed by surgical excision of the left adrenal mass. The biopsy subsequently revealed non-malignant adrenal tumor (pheochromocytoma). We are reporting successful treatment of rare case with RDS concomitantly presented with large adrenal pheochromocytoma. He was treated medically and surgically, the patient got recovery. RDS is a rare disease but we should consider it in patients with repeated benign lymphadenopathy. we are emphasizing that knowledge about RDS and its disease associations may be informative to the general medical community.

Keywords: Adrenal pheochromocytoma, Rosai–Dorfman syndrome, sinus histiocytosis

How to cite this article:
Mahwi TO, Khoshnaw N. Management of a patient with Rosai–Dorfman syndrome and large adrenal pheochromocytoma. Iraqi J Hematol 2018;7:45-7

How to cite this URL:
Mahwi TO, Khoshnaw N. Management of a patient with Rosai–Dorfman syndrome and large adrenal pheochromocytoma. Iraqi J Hematol [serial online] 2018 [cited 2023 Jan 27];7:45-7. Available from: https://www.ijhonline.org/text.asp?2018/7/1/45/225331

  Introduction Top

Sinus histiocytosis with massive lymph node (LN) enlargement was first described in 1969 by Rosai and Dorfman as a distinct clinicopathological entity.[1] It is characterized by nonmalignant proliferation of histiocytes (non-Langerhans histiocytes) and phagocytes in the LNs and extralymphatic sites.[2] Rosai–Dorfman syndrome (RDS) patients are usually asymptomatic or presented with mild symptoms, the median age of 39 years at presentation and more frequently found in male gender and African American than Asian backgrounds.[1],[2],[3] Clinical features include fever, painless LN enlargement, and increased leukocytes. The LN enlargement is usually found in the head, neck, cervical, axillary, and inguinal regions. Extralymphatic involvements are reported also, and the most common sites are a central nervous system, orbit, eyelids, skin (mainly in females), and gastrointestinal system and upper respiratory tract.[4],[5] Very rarely, some patients present with a painless maculopapular rash and bone infiltration with hepatosplenomegaly.[3],[6] The etiology of RDS is unknown, but many factors are described as immune dysfunction and viral infections such as parvovirus B19, EBV, and HHV-6 or may be associated with IgG4-related disorders and T-regulatory cell dysfunction. Genetic abnormality (SLC29A3) had been reported in a family with RDS. Patients with RDS are usually better to be sent to imaging a brain MRI to exclude CNS involvement.

LN biopsy reveals matted enlarged LNs with thick capsules, and in RDS, you may see LNs contain histiocytes that have an abundant cytoplasm which contains lymphocytes and plasma cells and this process called emperipolesis.[1],[2] Immunohistochemistry shows positive for CD68, CD163, and S100 and negative for CD1a and BRAF-V60E.[7],[8] The common differential diagnoses include tuberculosis, sarcoidosis, IgG4-related diseases,[9],[10],[11],[12],[13],[14] juvenile xanthogranuloma, Gaucher disease, histiocytic disorders, Hodgkin's/non-Hodgkin's lymphoma, and melanoma.

The treatments of RDS are usually variable which include surgical excision, or chemotherapy like alkylating agents, anthracyclines, Methotrexate, cladribine, and lenalidomide, or biological agents such as rituximab, retinoids, or steroid, and interferon, lastly topical cryotherapy, and radiotherapy also have been mentioned.[15],[16],[17],[18],[19]

The prognosis is like that one-fifth of patients got spontaneous regression, while two-thirds of them may have frequent relapses. Here in we describe successful treatment of rare case with RDS who presented with the large adrenal tumor. we are emphasizing that knowledge about RDS and its disease associations may be informative to the general medical community.

  Case Report Top

A 43-year-old male presented with bilateral inguinal swelling for a 3-year duration. The condition associated with intermittent mild fever, mild inguinal pain, and tenderness. Frequent fine-needle aspiration has been done, and the excision biopsy of the LNs revealed reactive hyperplasia; finally, one of the biopsies showed marked sinus histiocytosis with lymphophagocytosis (emperipolesis), with lymphoplasmacytic infiltration with S100 positivity [Figure 1]a,[Figure 1]b,[Figure 1]c. One year later, he developed headache, sweating, and attacks of raised blood pressure reaching >200/100 mmHg, hyperglycemia, and irritability, with weight gain and obesity. The patient investigated by ultrasonography and computerized tomography, revealing a well-defined mass between upper pole of the left kidney and spleen (79 mm × 83 mm) [Figure 2]a and right side inguinal LN enlargement of about 42 mm in diameter; the sonography of the neck showed enlarged LNs with reactive changes.
Figure 1: Right inguinal lymph node excision biopsy, stained with hematoxylin and eosin, under power (panel a) ×100, (panel b) x40, and (panel c) x20 magnification, showing sinus histiocytosis and characteristic emperipolesis

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Figure 2: (a) Computerized tomography of a 43-year-old male, with mass between spleen and left kidney showing an adrenal mass. (b) Panel B: Showing an abdominal scar

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On routine blood tests, there was high blood sugar and high alanine aminotransferase, while other blood tests were normal. The patient had been admitted to the medical unit for the control of his blood pressure and blood sugar for few days before preparation for surgical excision of the left adrenal mass on September 2015. The excised mass grossly looked like an encapsulated oval mass of 8.5 cm × 7 cm × 6 cm, weighed 200 g, adherent to the normal-looking adrenal gland of 6 cm. Microscopically, it consists of pleomorphic large polygonal cells arranged in nests bounded by delicate fibrovascular stroma. The cells had granular eosinophilic cytoplasm and moderate-to-severe nuclear pleomorphism, and mitosis cannot be found. Special stains such as AE1/AE3 and chromogranin A were positive with focal moderate positivity for synaptophysin. Finally, it was diagnosed as adrenal neoplasm (oncocytic pheochromocytoma with none malignant features). Postoperatively, blood pressure settled and his blood sugar stabilized over few months with diet regimen and using metformin as outpatient treatment [Figure 2]b.

  Discussion Top

RDS is rare disease and usually involves lymph-nodes, but may involve extra-nodular lesions.[1] The main way of treatment is surgical excision of the mass, steroid, chemotherapy, radiotherapy and others.[15],[16],[17],[18],[19] The severity of the high blood pressure and blood sugar before surgical removal of the adrenal mass indicate the high activity of sympathetic neuroendocrine activity of the pheochromcytoma. The response was dramatic after surgery; the patient still remained on Metformin and diet regime. We reviewed literature but we couldn't find significant relation between these two diseases. This condition needs more investigation to prove the relation, or it may be a coincidental presentation.

  Conclusion Top

Here, we are reporting a very rare case of RDS in a middle-aged Kurdish male who presented with recurrent LN enlargement and concomitant presentation with large adrenal mass with features of pheochromocytoma; the patient is treated medically and surgically with a very good response.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


We would like to thank Shorsh Hospital and Hiwa Hospital for performing pathological report and managing the case and the patient who gave us permission to publish his data.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.  Back to cited text no. 1
Gaitonde S. Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy: An overview. Arch Pathol Lab Med 2007;131:1117-21.  Back to cited text no. 2
Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity. Semin Diagn Pathol 1990;7:19-73.  Back to cited text no. 3
Lauwers GY, Perez-Atayde A, Dorfman RF, Rosai J. The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): Review of 11 cases. Hum Pathol 2000;31:380-5.  Back to cited text no. 4
Frater JL, Maddox JS, Obadiah JM, Hurley MY. Cutaneous Rosai-Dorfman disease: Comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case. J Cutan Med Surg 2006;10:281-90.  Back to cited text no. 5
McClellan SF, Ainbinder DJ. Orbital Rosai-Dorfman disease: A literature review. Orbit 2013;32:341-6.  Back to cited text no. 6
Detlefsen S, Fagerberg CR, Ousager LB, Lindebjerg J, Marcussen N, Nathan T, et al. Histiocytic disorders of the gastrointestinal tract. Hum Pathol 2013;44:683-96.  Back to cited text no. 7
Singhi AD, Montgomery EA. Gastrointestinal tract langerhans cell histiocytosis: A clinicopathologic study of 12 patients. Am J Surg Pathol 2011;35:305-10.  Back to cited text no. 8
Zhang X, Hyjek E, Vardiman J. A subset of Rosai-Dorfman disease exhibits features of IgG4-related disease. Am J Clin Pathol 2013;139:622-32.  Back to cited text no. 9
Tsang WY, Yip TT, Chan JK. The Rosai-Dorfman disease histiocytes are not infected by Epstein-Barr virus. Histopathology 1994;25:88-90.  Back to cited text no. 10
Noguchi S, Yatera K, Shimajiri S, Inoue N, Nagata S, Nishida C, et al. Intrathoracic Rosai-Dorfman disease with spontaneous remission: A clinical report and a review of the literature. Tohoku J Exp Med 2012;227:231-325.  Back to cited text no. 11
Mehraein Y, Wagner M, Remberger K, Füzesi L, Middel P, Kaptur S, et al. Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. J Clin Pathol 2006;59:1320-6.  Back to cited text no. 12
Luppi M, Barozzi P, Garber R, Maiorana A, Bonacorsi G, Artusi T, et al. Expression of human herpesvirus-6 antigens in benign and malignant lymphoproliferative diseases. Am J Pathol 1998;153:815-23.  Back to cited text no. 13
Ortonne N, Fillet AM, Kosuge H, Bagot M, Frances C, Wechsler J. Cutaneous Destombes-Rosai-Dorfman disease: Absence of detection of HHV-6 and HHV-8 in skin.J Cutan Pathol 2002 ;29:113-8.  Back to cited text no. 14
Purav P, Ganapathy K, Mallikarjuna VS, Annapurneswari S, Kalyanaraman S, Reginald J, et al. Rosai-Dorfman disease of the central nervous system. J Clin Neurosci 2005;12:656-9.  Back to cited text no. 15
Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, Velazquez-Vega J, Borja MJ, Rosenberg S, et al. Rosai-Dorfman disease of the central nervous system: Report of 6 cases and review of the literature. Medicine (Baltimore) 2014;93:165-75.  Back to cited text no. 16
Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-dorfman disease): Report of a case and literature review. Am J Hematol 2002;69:67-71.  Back to cited text no. 17
Forest F, N'guyen AT, Fesselet J, Metellus P, Bouvier C, de Paula AM, et al. Meningeal Rosai-Dorfman disease mimicking meningioma. Ann Hematol 2014;93:937-40.  Back to cited text no. 18
Symss NP, Cugati G, Vasudevan MC, Ramamurthi R, Pande A. Intracranial Rosai Dorfman disease: Report of three cases and literature review. Asian J Neurosurg 2010;5:19-30.  Back to cited text no. 19
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