Rosai-Dorfman disease. Extranodal sinus histiocytosis in three co-existing sites. A case report

A 73-year-old woman presented with mild anterior uveitis, ipsilateral optic neuropathy, and ipsilateral skin nodules. A compressive mass at the level of the orbital apex and sphenoid wing was found on cranial magnetic resonance imaging. Biopsy of the skin nodules revealed histopathologic evidence of sinus histiocytosis with massive lymphadenopathy, or Rosai–Dorfman disease (RDD). Systemic investigations failed to show any massive lymphadenopathy, making this a case of extranodal RDD. This is a salient case in that it proposes three simultaneous and separate sites of involvement by extranodal RDD. It also exemplifies that RDD should be a suspect diagnosis even in the absence of lymphadenopathy.     

Extranodal rosai-dorfman disease with cutaneous and periodontal involvement: a rare presentation

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) was first described as a distinct clinicopathological entity by Rosai and Dorfman in 1969. SHML is commonly characterized by painless cervical lymphadenopathy, and its clinical course is generally benign and self-limiting. This disorder commonly involves the lymph nodes but may secondarily involve the skin. However, purely cutaneous disease without lymphadenopathy or internal organ involvement rarely occurs. In the absence of the massive lymphadenopathy that is characteristic of RDD, the diagnosis of purely cutaneous RDD may be complicated by the rare, nonspecific clinical appearance of skin lesions and the broad histopathological differential diagnosis of this disorder. A high level of suspicion of this disease on the part of the clinician or pathologist is often required. We present a case of cutaneous RDD associated with involvement of periodontal tissue.     

淋巴结外Rosai-Dorfman病二例报告并文献复习

为了探讨淋巴结外Rosai-Dorfman病的病理学特征、临床表现、诊断、治疗及预后,对2例结外Rosai-Dorf-man病行HE和免疫组织化学染色观察,并对其进行随访。结果显示,光镜下见病灶内有大量含嗜酸性胞质的组织细胞,胞质中可见被吞噬的淋巴细胞、浆细胞或中性粒细胞,S-100和CD68染色( )。治疗方法多样,疗效及预后不同。初步研究结果提示,结外Rosai-Dorfman病是一种少见的组织细胞增生性病变,有一定的病理学特征。由于其临床表现多样,导致诊断较难,治疗效果不尽相同。     

Extranodal sinus histiocytosis with massive lymphadenopathy--a case report.

Sinus histiocytosis with massive lymphadenopathy involving organs other than the lymph nodes is a rare event. A case of SIIML presenting with multiple skin and subcutaneous nodules and multiple osteolytic lesions is described. A search of the Indian literature revealed many cases of nodal SIIML, but none of the reported cases had prominent extranodal involvement.     

Rosai-Dorfman病一例及文献分析

目的探讨Rosai-Dorfman病（RDD）的临床表现、诊断、组织特征及治疗。方法研究RDD1例,结合文献回顾分析。结果RDD1例,病理见大量淋巴细胞、浆细胞及组织细胞,并有吞噬淋巴细胞现象,免疫组化S-100强阳性。本病临床表现多样,易误诊。结论RDD是良性的组织细胞增生性疾病,预后主要取决于患者的免疫功能状态、淋巴结受累数目及结外器官受累部位,诊断主要依靠病理及免疫组化,治疗上以综合治疗为主,治疗后应随访。     

Usefulness of oral corticosteroid in Rosai-Dorfman disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign self-limiting disorder of unknown aetiology, which is frequently mistaken for lymphoma. There is no consensus as to the proper management of this disorder. In the past, potentially harmful treatments, such as antineoplastic drugs, have been advocated. We describe a 25-year-old woman with RDD who has had a remarkably favourable response to oral prednisolone therapy. Our observation strengthens the conclusions of previous case reports as to the effectiveness of corticosteroids in this condition. This article is aimed at creating awareness among clinical oncologists about this pseudolymphomatous disorder and emphasizing the therapeutic role of corticosteroids.     

颅内原发性Rosai-Dorfman病1例报道及文献回顾

背景与目的：Rosai-Dorfman病（Rosai-Dorfman disease,RDD）是一种少见的良性组织细胞增生性疾病,多位于淋巴结内,颅内RDD极少见。本文探讨颅内RDD的病理、诊断、鉴别诊断及治疗。方法：对1例颅内RDD病例进行病理形态观察和免疫组化检测,并对文献报道进行回顾。结果：本例患者病灶位于右侧额、颞部和基底池,肿物基底位于硬膜。病理和免疫组化见病变组织由数量不等的成熟淋巴细胞、浆细胞及S-100、CD68标记阳性的组织细胞构成,组织细胞内有吞噬淋巴细胞或淋巴细胞＂伸入现象＂。结论：原发性颅内窦组织细胞增生症是一种原发于中枢神经系统的罕见的良性组织细胞增生性疾病,缺乏特异性临床表现,确诊依赖病理学检查。因本例在颅内有多个病灶,手术难以全部切除,采用保守治疗包括使用激素效果良好。     

Lymphadenopathic type of Kaposi's sarcoma presenting with generalized petechial hemorrhages. A case report from Taiwan

An 8-year-old Taiwanese girl presenting with generalized petechial hemorrhages, severe thrombocytopenia and lymphadenopathy was found to have primary Kaposi's sarcoma of lymph node without cutaneous involvement. This patient is a first-documented case of lymphadenopathic type of Kaposi's sarcoma in Taiwan. The associated severe thrombocytopenia resulting in generalized petechial hemorrhages as the initial manifestation is also unique in this case.     

Indolent Lymphoproliferative T-Cell Disorders Associated With Gastrointestional Disease: Diagnostic Challenges and Outcomes

Lymphoproliferative diseases arise when the physiological mechanisms that control the proliferation of T and B lymphocytes are disrupted, resulting in an uncontrolled and autonomous increase in immune cells leading to lymphocytosis and lymphadenopathy, and often to the involvement of extranodal sites.The differential diagnosis of malignant T cell tumors involves other neoplasms and non–clonal T cell proliferations. Immunological markers are essential, as a first step, to distinguish between T-cell and non–T-cell disorders. It must be established based on the configuration of the genes of the TCR chain to rule out that the picture is not reactive to other underlying diseases. This clinical review and accompanying case reports highlight the diagnostic challenges associated with indolent lymphoproliferative T-cell disorders, which in many cases may represent the clinical manifestation of a single disease. Particularly we focus on gastrointestinal manifestations that could be expression either of lymphoproliferative disorder either of autoimmune disease either of both. The correct interpretation of the different clinical situations can help in the diagnostic and therapeutic process.     

A Rare Case of “Rosai–Dorfman Disease”

Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign proliferating histiocytic disorder, predominantly of lymphnodes with extranodal involvement also seen. We present a case of 35 years old female with history of multiple swellings in neck since 1 month duration. On examination patient had painless bilateral cervical lymphadenopathy. No other ENT manifestations noted. Lymphnode biopsy revealed SHML. Abdominal scan and chest X-ray was done which was normal. This case report highlights the clinical, histological aspects of SHML, Rosai–Dorfman disease.     

Primary non-Hodgkin’s lymphoma of the transverse colon presenting as dermatomyositis

Primary extranodal non-Hodgkin’s lymphoma of the transverse colon is a rare presentation of non-Hodgkin’s lymphoma or colonic neoplasm. Dermatomyositis is an autoimmune condition of the skin, muscle, and blood vessels that when associated with malignancy is a true paraneoplastic syndrome but is rarely associated with non-Hodgkin’s lymphoma. We present a case of primary non-Hodgkin’s lymphoma of the transverse colon diagnosed after the presentation of dermatomyositis and review the literature on dermatomyositis and hematologic neoplasm.     

Multifocal extranodal lymphomas: an expression of homing phenomenon.

The present study analyses 27 patients with primary lymphoma involving multiple extranodal sites. Eight patients were found to have multiple extranodal involvement at presentation while in 19 patients, relapse of disease was noticed at a different extranodal site without any other systemic spread. Malignant lymphomas of Waldeyer's ring were frequently associated with involvement of gastrointestinal, gonadal or cutaneous lesion. Primary gonadal lymphoma also showed a high incidence of cutaneous, pleura and bone involvement. The homing pattern of mucosa-associated lymphoid cells explains the multifocal extranodal lymphomas with site-specific tropism.     

Chest X-Ray Changes in A.I.D.S.

Pulmonary complications are common in A.I.D.S. patients, and Pneumocystis pneumonia is the most frequent. The typical pattern is a fine perihilar infiltrate going on to a more pronounced reticulonodular and sometimes to an alveolar filling pattern. This latter pattern may herald respiratory failure. Pneumocystis can cause other unusual patterns, especially when the infection is chronic or is modified by simultaneous infection by other organisms. Other bacterial pulmonary infections have an aggressive course. Tuberculosis, even with a second or subsequent infection, has the radiological changes of a primary infection. Malignancies are most commonly non-Hodgkin's lymphoma and Kaposi's Sarcoma. They have no characteristic appearances. The occurrence of hilar lymphadenopathy in A.I.D.S. patients without Kaposi's Sarcoma, non-Hodgkin's lymphoma, TB or Pneumocystis requires further investigation as nodes are not a feature of A.I.D.S. per se. Although occasionally seen with Pneumocystis pneumonia, lymphadenopathy is more normally seen with Kaposi's Sarcoma, non-Hodgkin's lymphoma or tuberculosis. Because prompt and accurate treatment is essential in the immune compromised patient management of pulmonary infiltrates is based on a tissue diagnosis, either transbronchial or percutaneous biopsy.     

Body cavity-based presentation of natural killer cell lymphoma

We describe an unusual case of a 31-year-old Mexican woman who presented with pleural and peritoneal effusions involved by Epstein-Barr virus-positive non-Hodgkin's lymphoma of natural killer (NK)-cell lineage. The patient had no symptoms that could be related to her nasal region, and physical examination and radiologic studies showed no evidence of lymphadenopathy, organomegaly, or other extranodal masses. Thus, this case clinically mimicked body cavity-based lymphoma. Extranodal NK/T-cell lymphoma of nasal type is the current designation for these neoplasms in the recently proposed World Health Organization classification of lymphoid neoplasms. These tumors previously have been referred to many other names, including lethal midline granuloma, midline malignant reticulosis, polymorphic reticulosis, angiocentric immunoproliferative lesion, and angiocentric lymphoma. Nasal-type NK/T-cell lymphomas typically involve the nasal region, but may involve other extranodal sites, such as skin and gastrointestinal tract. The malignant cytologic features and the presence of azurophilic granules within the cell cytoplasm observed in Wright-Giemsa-stained cytocentrifuge preparations led to immunophenotypic and molecular genetic studies that were essential in establishing the correct diagnosis. As demonstrated in the case reported, extranodal NK/T-cell lymphomas of nasal-type can be clinically aggressive and may be associated with paraneoplastic phenomena.     

Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphoma

The clinical picture is of aggressive high and intermediate grade lymphoma with extranodal presentation either as the first manifestation or during the course of HIV infection. The dramatic growth of tumors, leukopenia, opportunistic infections, and pre-existing AIDS-related problems of KS and chronic infections have made treatment extremely difficult. New regimens using short courses of chemotherapy, GM-CSF, and antiviral therapy have raised hopes that these measures will lead to improved survival.     

Esophageal metastasis from a peripheral lung carcinoma masquerading as a primary esophageal tumor

A 65-year-old man presented with progessive dysphagia, which proved to be the first clinical manifestation of a periperal lung carcinoma (secondary to a submucosal metatasis in the esophagus). The lung tumor, hidden by the diaphragm on chest x-ray, was not suspected until a thoracotomy was done. Although dysphagia is known to be the first manifestation of bron-chogenic carcinomas, such presentation in a case of a peripheral lung carcinoma has not been well described. This case is reported with a review of the literature for cases with dysphagia secondary to a metastatic tumor in the esophagus.     

Primary bone marrow lymphoma is a rare neoplasm with poor outcome: case series from single tertiary care centre and review of literature

Primary bone marrow lymphoma is a rare disease and remains undiagnosed due to deceptive clinical presentation. Here, we report four cases of primary bone marrow B‐cell non‐Hodgkin lymphoma, which presented with cytopenias without any lymphadenopathy or organomegaly. Bone marrow examination revealed large atypical B‐cells with a reactive T‐cell infiltrate with suppression of the normal hematopoietic elements. This lymphoma is known to have a poor prognosis. Inspite of treatment, two of our patients died during chemotherapy. Two patients relapsed, of which one showed an early relapse after two months and was put on an alternative regimen. The other patient relapsed twice at an interval of 4 and 5 years, respectively, following which he remained in remission for another 5 years and had recently shown a relapse for the third time. Review of literature revealed seven case series and 11 case reports of primary bone marrow lymphoma in the last five decades.     

Unusual presentation of extranodal peripheral T-cell lymphomas with multiple paraneoplastic features.

Three patients with extranodal peripheral T-cell lymphoma and a distinctive clinical presentation are described. They had acute onset of fever, weight loss, progressive liver failure, bleeding diathesis, pancytopenia, and myelodysplastic changes in the bone marrow. Each patient had one or more paraneoplastic complications: severe rhabdomyolysis with myoglobinuria and secondary renal failure, cutaneous vasculitis, gluten-sensitive enteropathy, polyserositis, and increased macrophages with hemophagocytic activity. They did not have peripheral lymphadenopathy. The complex clinical presentations simulated collagen vascular disorders, systemic infections, or severe liver disease rather than a malignant lymphoma. Routine histologic studies revealed a small population of lymphoma cells in the bone marrow, spleen, and liver. Immunophenotyping studies demonstrated their T-cell phenotype, and cytogenetic analysis showed the clonality in Patients 1 and 2; clonal T-cell receptor gene rearrangement was found in Patients 2 and 3. These studies should be considered in the evaluation of patients with constitutional symptoms, liver failure, coagulopathy, and pancytopenia even in the absence of peripheral lymphadenopathy.     

Synchronous mucosa-associated lymphoid tissue (MALT) lymphomas involving bilateral orbits and breasts: a rare clinical entity

Primary orbital and primary breast lymphomas comprise very small subgroups of extranodal lymphomas. Clinical presentation at both these sites together is extremely rare. We describe a case of bilateral orbital and bilateral breast mucosa-associated lymphoid tissue (MALT) lymphomas with bilateral pre-auricular lymph nodal metastasis. The case history, staging and management for this unusual entity are discussed.