Cases reported "Histiocytosis, Sinus"

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1/8. Primary intracerebral rosai-dorfman disease: a case report.

    A 45-year-old woman presented with an isolated, contrast-enhancing brain lesion in white matter of the right frontal lobe, preoperatively thought to be either a primary brain neoplasm or metastasis. The lesion was demonstrated by histology and immunohistochemistry to be Rosai-Dorfman disease. central nervous system (CNS) manifestations of this disease are rare. There have been 27 cases of intracranial involvement reported previously. All of them have been dural-based, where the disease clinically and radiologically resembles meningioma. To our knowledge, this is the first case of an isolated intraparenchymal CNS lesion without dural attachment, where the clinical and radiological features resembled an intraparenchymal glial neoplasm, lymphoma or metastatic tumor.
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2/8. Coexistence of localized Langerhans cell histiocytosis and cutaneous Rosai-Dorfman disease.

    Rosai-Dorfman disease (RDD; sinus histiocytosis with massive lymphadenopathy) and Langerhans cell histiocytosis (LCH) are two different yet pathogenetically related histiocytic disorders. While systemic and localized forms have been identified in both diseases, each has its own characteristic histological, immunohistochemical and ultrastructural profile. Rarely, either RDD or LCH can also occur in the context of certain malignant neoplasms. However, the coexistence of RDD and LCH has never been described. We report a case of cutaneous RDD in which a focus of LCH was found. Clinical and laboratory examinations revealed no evidence of extracutaneous involvement of RDD or LCH. We believe that this is the first report of such a coexistence, and the possible pathogenesis is discussed.
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3/8. lung cancer and Rosai-Dorfman's disease. A clinicopathological study.

    CASE REPORT: A 60-year-old female patient underwent craniotomy for a cerebral lesion in the frontoparietal lobe. Histologically, it turned out to be a metastasis from an adenocarcinoma. The primary tumor was found in the upper lobe of the left lung. The patient had whole brain radiation therapy only, the lung tumor was not treated. 4 years later, she presented with enlarged cervical lymph nodes. A biopsy showed dilated sinuses filled with histiocytes, but no tumor cells. The diagnosis of sinus histiocytosis with massive lymphadenopathy was made (Rosai-Dorfman's disease). Without any treatment, the swelling resolved completely a few weeks later. Similar episodes were observed several times in the following years. The patient died more than 7 years after the diagnosis of a metastasizing lung cancer due to pneumonia. CONCLUSION: In a patient with a pulmonary neoplasm and suspected supraclavicular lymph node spread, Rosai-Dorfman's syndrome should be considered as a rare differential diagnosis.
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4/8. Salivary gland manifestations of sinus histiocytosis with massive lymphadenopathy: fine-needle aspiration cytology findings. A case report.

    Sinus histiocytosis with massive lymphadenopathy (SHML or Rosai-Dorfman disease) is a nonneoplastic, usually self-limiting disease. Alhough it affects all age groups, it is more commonly seen in young males in their first or second decades. The disease primarily manifests as painless lymphadenopathy of cervical region, but other nodal groups and extranodal sites may also be affected. Seldom SHML may involve the salivary glands. We present the cytologic features and differential diagnoses of one such case.A 45-yr-old woman presented with an enlarged submandibular gland on the left side with ipsilateral cervical lymphadenopathy that had been persisting for 1 mo. The gland and the two enlarged nodes measured 2.5 x 2.5 cm(2) each and were firm in consistency. Clinically, tumor of the salivary glands was suspected. Fine-needle aspiration (FNA) smears showed moderate cellularity, with large histiocytes dispersed in the background of intense lymphoplasmacytic infiltrate. These histiocytes showed lymphophagocytosis. Isolated stromal fragments consisting of fibrocytes were seen separate from salivary acinar clusters on repeat aspirations. The diagnosis of SHML involving left submandibular gland and ipsilateral lymph nodes was returned.When analyzed in the context of clinical findings (laboratory data), the cytologic features of SHML involving salivary gland could be differentiated from those of malignancies and other benign lesions, especially Kuttner's tumor of the submandibular gland, which mimics neoplasm clinically.
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5/8. Rosai-Dorfman disease of the testis: an unusual entity that mimics testicular malignancy.

    A 47 year old Chinese man with diabetes mellitus and previously treated pulmonary tuberculosis presented with painless right testicular enlargement of 1 month's duration. He underwent an orchidectomy for presumed testicular neoplasm corroborated clinicoradiologically. Histological examination of the testicular mass revealed an inflammatory lesion comprising lymphocytes, plasma cells and sheets of pale staining histiocytes, some containing lymphocytes within their ample cytoplasm, suggestive of emperipolesis. S100 immunohistochemistry stained the histiocytes, while ultrastructural examination confirmed emperipolesis. A diagnosis of Rosai-Dorfman disease was made, an exceedingly rare testicular lesion. Clinicoradiological findings mimicked a neoplasm, while the inflammatory histological appearances with occasionally discerned multinucleated cells raised the possibility of xanthogranulomatous orchitis. Tuberculous orchitis was excluded through negative Ziehl-Neelsen stains and PCR for mycobacterium, while seminoma, which sometimes features a predominant inflammatory component obscuring neoplastic cells, was excluded by absent immunostaining for placental alkaline phosphatase and CD117.
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6/8. Cutaneous Rosai-Dorfman disease.

    Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a benign idiopathic histiocytic proliferative disorder that commonly involves the lymph nodes but secondarily may involve the skin. However, purely cutaneous disease without lymphadenopathy or internal organ involvement rarely may occur. We present case reports of three patients who presented with asymptomatic nonspecific enlarging skin nodules without evidence of lymphadenopathy or internal disease. Histopathologic examination of skin lesions in all patients showed proliferation of large histiocytes with phagocytosed inflammatory cells characteristic of Rosai-Dorfman disease. However, the diagnoses of dermatofibroma, other spindle cell neoplasm, infectious granulomatous process, and other xanthohistiocytic proliferations were also considered due to the presence of storiform spindle cells and foamy cells in the first case. One patient experienced regression during a course of oral steroids, while another patient cleared spontaneously. In the absence of massive lymphadenopathy characteristic of Rosai-Dorfman disease, the diagnosis of purely cutaneous Rosai-Dorfman disease may be complicated by the rarity, non-specific clinical appearance of skin lesions, and broad histopathological differential diagnosis of this disorder. A high index of suspicion of the clinician and pathologist is often required.
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7/8. Hodgkin's disease and sinus histiocytosis with massive lymphadenopathy-like changes.

    Sinus histiocytosis with massive lymphadenopathy (SHML) is a disorder of unknown origin which is only infrequently associated with lymphoid neoplasms. We report the first two cases of Hodgkin's disease with simultaneous SHML-like changes in the same lymph node.
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8/8. Soft tissue Rosai Dorfman disease mimicking inflammatory pseudotumor: a diagnostic pitfall.

    Rosai Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), may be a difficult diagnosis to make, especially in extranodal sites. With soft tissue involvement the characteristic diagnostic features of large histiocytic cells with emperipolesis may be overshadowed by a fibroinflammatory component. In these cases it is easy to confuse this lesion with reactive nodules and benign and malignant neoplasms. We report a case in which soft tissue SHML was confused with an inflammatory pseudotumor. Only after review, when other extranodal sites became apparent, was the correct diagnosis made. Pitfalls in the diagnosis of soft tissue SHML are discussed.
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