羅賽-朵夫曼病合併主動脈旁及後腹腔淋巴結病變:罕見案例報告與文獻分析

黃品叡 1、黃彥端1、洪啟峰 1、劉憲2、周詠欽 3,4、沈正煌 1、林昌德 1、鄭明進 1

1 戴德森財團法人嘉義基督教醫院 外科部 泌尿科

 2 戴德森財團法人嘉義基督教醫院 外科部 一般外科

3天主教聖馬爾定醫院 泌尿科

4亞洲大學 食品營養與保健生技學系

Rosai-Dorfman disease with para-aortic and unilateral retroperitoneal lymphadenopathy in breast cancer: A rare case report and literature review

Pin-Jui Huang¹, Yen-Tuan Huang¹, Hung-Chi Fang¹, Xian Liu², Yeong-Chin Jou^1,3,
Cheng-Huang Shen¹, Chang-Te Lin¹, Ming-Chin Cheng¹

¹Devision of Urology, Department of Surgery, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Chia-Yi, Taiwan

²Devision of General Surgery, Department of Surgery, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Chia-Yi, Taiwan

³Department of Urology, St. Martin De Porres Hospital, Chia-Yi, Taiwan

⁴Department of Food Nutrition and Health Biotechnology, Asia University

Introduction:

    Rosai-Dorfman disease is described as a rare and benign histiocytic disorder most frequently affecting cervical lymph node in children, young adults. In this article, we sincerely report a case who was a victim of left breast cancer with stage IIB, status post operation and adjuvant chemotherapy. Para-aortic and unilateral retroperitoneal lymphadenopathy were noticed by follow-up abdominal computer tomography (CT), and the patient then underwent laparoscopic dissection of the enlarged lymph nodes. However, the Rosai-Dorfman disease (RDD) was confirmed by final pathology, and there was no evidence of nodal metastasis. The literature of Rosai-Dorfman disease is also reviewed in this article.

Case presentation:

    This 58-year-old postmenopausal woman had past history of hypertension and type 2 diabetes mellitus (HbA1c: 6.3%). She was a victim of left breast cancer, with pathological stage IIB. She had received curative surgery and adjuvant chemotherapy. During the annual outpatient office follow-up, abdominal computed tomography (CT) showed an 1.3cm enlarged lymph node located at right infra-renal space. Another enlarged lymph node was also noticed at celiac/left grastric area with diameter 2.7cm in size. Positron emission tomography / Computed tomography (PET-CT) was arranged for further survey, and the result showed one hypermetabolic celiac node and one hypermetabolic node at right infra-renal space. The finding was compatible with previous CT finding. Breast cancer with non-regional lymph node metastasis was impressed. Operation of laparoscopic excision of the pathological lymph nodes at celiac/left gastric area and right infra-renal space was performed. However, the final pathological review reported as Rosai-Dorfman disease with sinus histiocytosis and no sign of malignancy. Drainage of vacuum ball was removed on postoperative day 3. Under stable condition, the patient was discharged on postoperative day 5.

Discussion:

    The classic clinical presentation of Rosai-Dorfman disease (RDD) is bilateral painless cervical lymphadenopathy. Intermittent fever, night sweating, and weight loss might also present in some cases. Extranodal involvement was reported in 43% of RDD patient, including skin, upper respiratory tract, and bone. For establishing diagnosis of Rosai-Dorfman disease, positron emission tomography (PET) / computed tomography (CT) was suggested for all suspicion of this disease. Then biopsy of lesion tissue was recommended for definitive diagnosis. According to the current literature, there is no uniform treatment has been recommended for RDD. The choice of treatment is based on the individual clinical circumstances. Available treatment that had been reported included observation, surgery, steroid, sirolimus, chemotherapy, immunomodulatory therapy, target therapy, radiotherapy. Overall, the most common first line therapeutic modality was surgical excision. The relapse rate is 33%. For patient with asymptomatic RDD , observation is a suitable strategy. Surgery for RDD is mainly for tissue proof, but resection could be a curative treatment for unifocal disease, and debulking may be warranted for upper airway obstruction, spinal cord compression, or large lesions causing end-organ compromise. Long-term remission after resection of lesion was reported in isolated intracranial disease. The most effective treatment of cutaneous RDD is surgical excision.

In our case, laparoscopic lymph node dissections was performed for proof of lesion tissue. The final pathological review was categorized as sporadic classic nodal subtype of RDD based on Schematic classification of Rosai-Dorfman disease. Such type of RDD is usually self-limited and has a good outcome, with spontaneous remission reported in up to 50% of cases. However, it played a role of diagnostic mimicker in case of breast cancer. Breast cancer with suspicious nodal metastasis was impressed initially in our case. Reviewing literature, Hoffman et al. admitted that cases of RDD are rare, so it is not surprising that some may be misdiagnosed as an inflammatory process or other metastatic neoplasm. In Cassandra et al, tissue proof such as fine needle aspiration or biopsy is still needed if neoplasm was under suspicion.

Conclusion:

    Rosai-Dorfman disease (RDD) is an uncommon disease, and sporadic classic nodal subtype has a benign outcome with an estimated 50 % self-submission rate. To exclude other suspecting disease such as inflammatory disease, positron emission tomography (PET) / computed tomography (CT) is the best examination for exclusion of such disease. Unfortunately, it is hard to tell between RDD and metastatic lymph node via PET-CT due to similar presentation in both groups. Fine needle aspiration or biopsy is still needed if neoplasm was suspected.