|Year : 2016 | Volume
| Issue : 1 | Page : 40-43
Rosai Dorfman Disease of a ten month Saudi Infant: A case report
Ali S Al-Qahtani1, Mubarak Al-Shraim2
1 Associate Professor and Consultant ENT/Head & Neck Surgeon, Department of Ear, Nose, Throat and Head & Neck Surgery, College of Medicine, King Khalid University, Abha, Kingdom of Saudi Arabia
2 Associate Professor of Pathology, Department of Pathology, College of Medicine, King Khalid University, Abha, Kingdom of Saudi Arabia
|Date of Web Publication||7-Aug-2020|
FKSU, FACS, FISQua Ali S Al-Qahtani
Vice Dean for Academic Development and Quality, College of Medicine, King Khalid University, P.O. Box 3877, Abha 61481
Kingdom of Saudi Arabia
Source of Support: None, Conflict of Interest: None
Rosai-Dorfman Disease is a rare disorder. We report a case of ten-month Saudi male infant who presented with persistent painless left neck mass for the previous three months, accompanied by fever, odynophagia, night sweats and weight loss. Physical examination showed enlarged left upper cervical lymph node measuring up to five to seven cm and filling up the gap anterior to sternocleidomastoid muscle and angle of mandible. It was cystic, fluctuant, semi-mobile, non tender. Computed tomography (CT) neck with IV contrast were performed, which revealed non enhancing hypodense lymph node at upper cervical region. Lymph node was surgically removed. Histopathology confirmed Rosia-Dorfman Disease. The patient made good recovery and was discharged on oral prednisolone 10mg daily for one month. A one -year follow-up did not reveal any recurrences.
Keywords: Rosai-Dorfman, Histiocytosis, odynophagia, lymphadenopathy
|How to cite this article:|
Al-Qahtani AS, Al-Shraim M. Rosai Dorfman Disease of a ten month Saudi Infant: A case report. King Khalid Univ J Health Scii 2016;1:40-3
|How to cite this URL:|
Al-Qahtani AS, Al-Shraim M. Rosai Dorfman Disease of a ten month Saudi Infant: A case report. King Khalid Univ J Health Scii [serial online] 2016 [cited 2020 Nov 28];1:40-3. Available from: https://www.kkujhs.org/text.asp?2016/1/1/40/291598
| Introduction|| |
Rosai-Dorfman Disease (RDD) is a rare, benign histocytic disorder described first by Rosai and Dorfman in 1969. It is characterized by painless bilateral neck lymph node enlargement associated with fever. Generally seen in younger patient, but any age group can be affected. The cause of RDD is not fully understood, and the diagnosis can be missed due to its rarity and histological similarity to other diseases. Treatment strategies depend on vital organs involvement and in some cases, may cause mortality. We report a first case of a Saudi infant boy with Rosai-Dorfman Disease.
| Results|| |
A 10-month-old Saudi boy presented with three month history of painless left neck mass which increased gradually accompanied by fever, odynophagia, night sweats and weight loss. No previous medical or surgical problem before. Physical examination on admission the vital signs were within normal limits. Examination of the neck revealed left neck mass measuring 5x7 cm at the upper cervical lymph node which is firm and non-tender. No other contributory lymph node enlargement. Endoscopic examination of nose and nasopharynx was normal. Chest clear examination of abdomen revealed no hepatosplenomegaly confirmed by ultrasound. Laboratory investigation revealed Hb12.3 gm/dl, WBC 6.4 10/ul, ESR, C-reactive protein and urea creatinine electrolyte were within normal limit. Chest x-ray was normal. Computed tomography (CT) of neck with IV contrast were performed, which revealed nonenhancinghypodense lymph node at upper cervical region measuring 7x5cm no calcification and no cystic degeneration [Figure 1]. Patient was admitted to the Hospital for excisional biopsy [Figure 2].
|Figure 1: Axial CT image wit IV contrast revealed nonenhancinghypodense lymph node at upper cervical region measuring 6×4 cm.|
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|Figure 2: Post Excision of upper cervical lymph node region measuring 6×4 cm.|
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Histopathology of lymph node showed enlarged lymph node in which there was mild capsule and pericapsular inflammation and fibrosis. The lymph node architecture is altered by prominent sinuses dilation filled by large histiocytes containing intact lymphocytes, histopathologic phenomena known as emperipolesis [Figure 3]. The histiocytes were large, round with vesicular nuclei and prominent nucleoli. The intersinusoidal spaces contain lymphocytes and plasma cells surrounded high endothelial venules [Figure 4]. Immunohistochemical stain of S-100 revealed diffuse and strong cytoplasmic staining of numerous histiocytes [Figure 5].
|Figure 3: Light microscopic examination showing enlarged lymph node, in which, there are mild capsule, pericapsular inflammation and fibrosis. The lymph node architecture is altered by prominent sinuses dilation (asterisks) filled by large histiocytes containing intact lymphocytes, the histopathologic phenomena known as emperipolesis. (HE, 10× magnification).|
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|Figure 4: Light microscopic examination showing large histiocytes with round vesicular nuclei and prominent nucleoli (black arrows). Histiocytes appear containing many lymphocytes (white arrow heads). The intersinusoidal spaces contain lymphocytes (black arrow heads) and plasma cells (white arrows) surrounding high endothelial venules. (HE, 40× magnification).|
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|Figure 5: Photomicrograph of S100 immuno-histochemical stain showing histocytes containing intracytoplasmic small lymphocytes “emperipolesis”. (original magnification 200×). Arrow indicates small lymphocyte inside the cytoplasm of the histocyte|
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Immunohistochemical staining for leukocytes common antigen (LCA, CD45) was positive for the lymphocytes in the background which showed a mixed population of B and T lymphocytes confirmed by CD20 and CD3 immunoreactivity.
The patient was started on oral prednisolone10mg daily for a month. His symptoms greatly improved within the week following surgery and he was discharged home in good condition. After a one-yearfollow-up period showed no signs of recurrence of the disease.
| Discussion|| |
Rosia-Dorfman Disease is a rare benign systemic histoproliferative disorder characterized by massive cervical lymphadenopathy with fever, extranodal involvement has been documented in 43% of cases. It is most frequently seen in children and young adult. The sixty seven percent of all cases occur in less than ten year of age. RDDis more common in male than female with high incidence in Africa and West Indian desent.
The most common clinical presentation is bilateral painless neck lymphadenopathy with fever, night sweats and weight loss. There are three types of histolytic proliferative disorder type 1 also known as Langerhan cell histiocytosis is due to reactive proliferation of Langerhan cells, type 2a is due to reactive proliferation of dermal dandrocytes type, type 2b due to reactive proliferation of histiocytes, and type 3 is due to malignant proliferation of histiocytes. Rosai-Dorfman Disease (RDD) also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is a very rare disorder belonging to type 2b. The differential diagnosis include lymphoma, nasopharyngeal carcinoma and granulomatous diseases such as Wagner’s granulomatosis, sarcoidosis and syphilis. The diagnosis is confirmed by histopathologic examination by demonstration of characteristic sinus histocytosis with lymphocytophagocytosis. Laboratory finding are non specific. Leukocytosis, 88.5% have an elevated erythrocyte sedimentation rate, 83% have a polyclonal elevation of immunoglobulin G, and 65% have anemia. The morphologic findings include pericapsular fibrosis and dilated sinuses heavily infiltrated with large histocyte, lymphocyte and plasma cells are diagnostic. The treatment in most of the cases are not necessary but some patient may require surgery which is limited to biopsy and debulking may be required in patient with vital organ involvement. An ideal treatment has yet to be identified. Systemic corticosteroid appears to be the most effective and found to be helpful in decreasing nodal size and symptoms., In our case we started postoperative small dose of prednisolone 10 mg for one month.
The symptoms greatly improved and he was discharged home in good condition. After the one year follow-up period showed no signs of recurrence of the disease.
| Conclusions|| |
Rosai-Dorfman Disease is a rare idiopathic disease, and mostly involve head and neck region with massive cervical lymphadenopathy with or without extranodal involvement, increase awareness of otorhinolaryngologist, pathologist and other clinician to be familiar of manifestation of RDD is important.
Conflict of interest: None, and is not funded by any drug company.
| References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]