A case report of nasopharyngeal inflammatory myofibroblastoma in infant / 临床耳鼻咽喉头颈外科杂志

Inflammatory myofibroblastic tumor （IMT） is a rare mesenchymal soft tissue tumor characterized by borderline or low-grade malignancy. It is rare childhood tumor with an average age of onset of 10 years old. It is even rarer in infants and toddlers, and the etiology and pathogenesis of this tumor are still unclear. The clinical presentation of IMT is non-specific and are related to the location of the tumor. When the tumor compresses adjacent organs, it can cause pain and functional impairment. According to the current literature, IMT is most commonly found in the digestive and respiratory systems, but also occasionally occur in the genitourinary system, head and neck, and limbs. At present, there have been no reports of nasopharyngeal IMT involving nasal cavity of infants and toddlers at home and abroad.This article reports a case of a massive inflammatory myofibroblastic tumor involving the nasal cavity and nasopharynx in an infant. Plasma-assisted minimally invasive surgery was performed through multiple surgical approaches and achieved satisfactory therapeutic results. This case report may provide valuable reference for the treatment of similar diseases.

Mammary myofibroblastoma: a clinicopathological analysis of fifteen cases / 中华病理学杂志

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of breast myofibroblastoma. Methods: The clinicopathological data and prognostic information of 15 patients with breast myofibroblastoma diagnosed at the Department of Pathology of the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China from 2014 to 2022 were collected. Their clinical characteristics, histological subtypes, immunophenotypes and molecular characteristics were analyzed. Results: There were 12 female and 3 male patients, ranging in age from 18 to 78 years, with a median and average age of 52 years. There were 6 cases in the left breast and 9 cases in the right breast, including 12 cases in outer upper quadrant, 2 cases in inner upper quadrant and 1 case in outer lower quadrant. Most of the cases showed a well-defined nodule grossly, including pushing growth under the microscope in 13 cases, being completely separated from the surrounding breast tissue in 1 case, and infiltrating growth in 1 case. Among them, 12 cases were classic subtype and composed of occasional spindle cells with varying intervals of collagen fiber bundles; eight cases had a small amount of fat; one case had focal cartilage differentiation; one case was epithelioid subtype, in which epithelioid tumor cells were scattered in single filing or small clusters; one case was schwannoma-like subtype, and the tumor cells were arranged in a significant palisade shape, resembling schwannoma, and one case was invasive leiomyoma-like subtype, in which the tumor cells had eosinophilic cytoplasm and were arranged in bundles, and infiltrating into the surrounding mammary lobules like leiomyoma. Immunohistochemical studies showed that the tumor cells expressed desmin (14/15) and CD34 (14/15), as well as ER (15/15) and PR (15/15). Three cases with histologic subtypes of epithelioid subtype, schwannoma-like subtype and infiltrating leiomyoma-like subtype showed RB1 negative immunohistochemistry. Then FISH was performed to detect RB1/13q14 gene deletion, and identified RB1 gene deletion in all three cases. Fifteen cases were followed up for 2-100 months, and no recurrence was noted. Conclusions: Myofibroblastoma is a rare benign mesenchymal tumor of the breast. In addition to the classic type, there are many histological variants, among which the epithelioid subtype is easily confused with invasive lobular carcinoma. The schwannoma-like subtype is similar to schwannoma, while the invasive subtype is easily misdiagnosed as fibromatosis-like or spindle cell metaplastic carcinoma. Therefore, it is important to recognize the various histological subtypes and clinicopathological features of the tumor for making correct pathological diagnosis and rational clinical treatment.

A case of Crohn's disease combined with inflammatory myofibroblastoma of abdominal wall / 中南大学学报(医学版)

Inflammatory myofibroblastoma (IMT) is a rare solid tumor, and its etiology and pathogenesis are unclear. Crohn's disease is a non-specific intestinal inflammatory disease. The clinical manifestations, laboratory examinations, and imaging examinations of IMT are not specific, making diagnosis difficult. A case of Crohn's disease combined with IMT of abdominal wall was admitted to the Department of Gastroenterology at the Third Xiangya Hospital, Central South University, on Nov. 21, 2017. This patient was admitted to our hospital because of repeated right lower abdominal pain for 4 years. A 6 cm×5 cm mass was palpated in the right lower abdomen. After completing the transanal double-balloon enteroscopy and computed tomographic enterography for the small intestinal, the cause was still unidentified. The patient underwent surgery due to an abdominal wall mass with intestinal fistula on Sept. 12, 2018 and recovered well currently. According to histopathology and immunohistochemistry, he was diagnosed with Crohn's disease combined with IMT. Up to July 2020, the patients still took azathioprine regularly, without abdominal pain, abdominal distension, and other discomfort, and the quality of his life was good.

Tumor miofibroblástico inflamatorio del colon / Inflammatory Myofibroblastic Tumor of the Colon

RESUMEN Introducción: Los tumores miofibroblásticos son tumores benignos de origen mesenquimal, de etiología incierta, y frecuente en niños y jóvenes. Objetivo: Describir un nuevo caso de tumor miofibroblástico abdominal. Caso clínico: Se presenta una paciente de 20 años con antecedentes de salud con dolor en abdomen derecho, se realiza tomografía de abdomen contrastada que informa una masa hiperdensa no homogénea en flanco derecho. Se realiza resección quirúrgica del tumor y el estudio anatomopatológico informa un tumor miofibroblástico inflamatorio. Su pronóstico es favorable porque es una entidad benigna y no metastiza. Conclusiones: Los tumores miofibroblásticos de colon son infrecuentes, benignos, que al diagnosticarlos deben resecarse ampliamente para evitar la recidiva(AU)

ABSTRACT Introduction: Myofibroblastic tumors are benign tumors of mesenchymal origin, of uncertain etiology, and frequent in children and young people. Objective: To describe a new case of abdominal myofibroblastic tumor. Clinical case: The case is presented of a 20-year-old female patient with a health history and pain in the right abdomen. Contrasted abdominal tomography is performed, which shows an inhomogeneous hyperdense mass in the right flank. Surgical resection of the tumor was performed. The pathological study reported an inflammatory myofibroblastic tumor. Its prognosis is favorable because it is a benign entity that does not metastasize. Conclusions: Colon myofibroblastic tumors are infrequent, benign, and when diagnosed, they must be widely removed to avoid recurrence(AU)

Tumores óseos benignos osteolíticos / Benign osteolytic bone tumors

INTRODUCCIÓN: Los tumores óseos primarios son raros. La localización, extensión y el carácter lítico dificultan el tratamiento aunque el comportamiento biológico sea benigno. OBJETIVO: Valorar las características y tratamientos de los tumores óseos benignos primarios líticos activos o agresivos tratados en el Hospital El Cruce. Comparar la resección realizada con la sugerida por los criterios de Enneking. MATERIAL Y MÉTODOS: se evaluaron historias clínicas y ateneos postquirúrgicos de pacientes con diagnóstico de tumores músculoesqueléticos atendidos en el Servicio de Ortopedia y Traumatología del Hospital El Cruce desde agosto de 2008 hasta junio de 2017. Se utilizaron los criterios de Ennekingde comportamiento biológico y resección. RESULTADOS: Se evaluaron 26 pacientes con diagnóstico de tumor benigno lítico activo o agresivo sobre un total de 124 tumores musculoesqueléticos primarios operados. Un paciente se reclasificó como maligno en la resección completa, por lo que se consideraron 25. Se trató de 16 mujeres, 9 varones, edad media 28,4.La localización fue: 13 miembro inferior (1coxal, 7 fémur, 3 tibia y 2 calcáneo), 9 miembro superior (1 húmero, 2 radio, 2 cúbito, 3 metacarpianos y 2 falanges) y 3 columna (1 cervical, 1 torácica y 1 sacra). Un paciente tuvo localización múltiple en la mano. Según Enneking 10 eran activos y 13 agresivos. Histología: 12 Tumores de células gigantes (TCG), 5 condromas, 4 quiste óseo simple, 3 quiste óseos aneurismáticos y 1 hemangioma. La resección fue intralesional en 16, en 3 marginal y en 6 amplia (1 amputación). La media de días de estada fue 7 días (0 a 33), 5 internación en UTI. La reconstrucción se hizo con: Injerto de Banco 7, Injerto de cresta 7, Injerto M.sup 3, Prótesis 2, Peroné vascularizado 2, Sustituto óseo y cemento en 1. Sin reconstrucción en 2. Dos tuvieron adyuvancia con denosumab. Once tuvieron complicaciones. Hubo un óbito. En 18/25 la resección realizada coincidió con los criterios de resección (Enneking). CONCLUSIONES: A pesar de la benignidad histológica, los tumores líticos primarios requirieron procedimientos complejos, con un 40 % de complicaciones. En el 72% la resección realizada concordó con los criterios de resección de Enneking.

INTRODUCTION: Primary bone tumors are rare. The localization, extension and lytic character make the treatment difficult even though the biological behavior is benign. OBJECTIVES: Assess the characteristics and treatments of active or aggressive benign primary lytic bone tumors treated in the El Cruce hospital. Compare the resection made with the one suggested by Enneking criteria. MATERIALS AND METHODS: Clinical records and postsurgical grand rounds were evaluated on patients diagnosed with musculoskeletical tumors treated in the Orthopedics and Traumatology Service of Hospital El Crucefrom August 2008 to June 2017. Enneking's criteria based on biologic behavior and resection were used. RESULTS:Twenty-six patients diagnosed with active or aggressive lytic bone tumor were evaluated out of a total of 124 operated primary musculoskeletal tumors. A patient was reclassified as malignant on the complete resection, therefore 25 were considered. There were 16 women, 9 men; average age was 28.4.The tumor localization was: 13 in lower extremity (1 coxal, 7 femoral, 3 tibial and 2 in calcaneus), 9 in upper extremity (1 humeral, 2 radial, 2 ulnar, 3 metacarpal y 2 in phalanges) and 3 in spine (1 cervical, 1 thoracic y 1 sacral). A patient had multiple localizations in his hand. Based on Enneking, 10 were active and 13 were aggressive. Histology: 12 giant-cell tumors (GCT), 5 chondromas, 4 simple bone cysts, 3 aneurysmal bone cysts and 1 hemangioma. The resection was intralesional in 16, marginal in 3 and ample in 6 (1 amputation). Mean hospitalization stay was 7 days (0 to 33), 5 days in ICU. The reconstruction was made with: draft from banks in 7, drafts from crests in 7, draft from upper extremity in 3, prosthesis in 2, vascularized fibula in 2, bone substitute and cement in 1. No reconstruction in 2.Two had adjuvant therapy with denosumab. Eleven had complications. There was one death. In 18 out of 25, the resection matched the resection criteria (Enneking). CONCLUSIONS: Despite the histological benignity, primary lytic tumors required complex procedures and there were 40% complications. In72%, the resection matched the resection criteria (Enneking).

Myofibroblastoma of the breast

@#Introduction: Myofibroblastoma is a rare benign mesenchymal tumour arising from the stromal elements of the breast tissue. Histopathological variants such as classic, cellular, collagenous / fibrous, lipomatous, infiltrative, myxoid and epithelioid have been identified. Most myofibroblastomas are immunoreactive for CD34, actin, CD10 and desmin, usually express oestrogen receptor (ER), progesterone receptor (PR) and variably express androgen receptor (AR). Case report: We report a case of myofibroblastoma in an octogenarian male presenting with painless solitary breast lump. Mammography (digital tomosynthesis) and ultrasound showed a well-circumscribed hyperdense mass and hypoechoic, solid, oval mass with peripheral vascularity respectively. Patient underwent wide local excision. Discussion: Diverse characteristics of myofibroblastoma on imaging necessitates histopathological analysis for an accurate diagnosis. Myofibroblastoma are often confused with fibroadenomas due to the benign imaging characteristics and with malignant neoplasia due to their wide morphological spectrum. Surgical excision is considered curative.

A Case of Mammary-Type Myofibroblastoma of the Tongue / 대한이비인후과학회지

A mammary-type myofibroblastoma is a rare benign mesenchymal neoplasm composed of spindle cells initially described to occur in the breast. However, they also arise at extra-mammary sites including the inguinal area, breast, chest wall/axilla, trunk, upper and lower extremities, and head and neck regions. In particular, mammary-type myofibroblastoma of the head and neck are extremely rare and may occur at any age. Immunohistochemically, it is typically CD34 and desmin positive. Currently, complete excision is considered as the first line treatment and recurrence of the tumor is rare. We experienced a 41-year-old man who visited with 1 cm sized firm mass of the tongue. The mass was resected and tissue biopsy revealed a diagnosis of mammary-type myofibroblastoma. Herein we report a rare case of mammary-type myofibroblastoma of the tongue with a review of the literature.

Clinicopathologic evaluation of myofibroblastoma: A study in two hospitals

OBJECTIVE: To report various anatomic locations and clinical characteristics of pathologically proven myofibroblastoma in Koran patients. METHODS: Pathologic reports of patients who underwent surgeries at two centers between April 2003 and March 2016 were retrieved from the electronic medical record system of the hospital. Pathologic reports were included after performing a search using the keyword “myofibroblastoma”. RESULTS: The cohort consisted of 11 subjects and included eight female and three male individuals. The patients' ages ranged from 9 to 66 years. Tumors were located in the vagina in three patients and presented in the breast in seven patients. One case presented with an abdominal mass. The tumors ranged in mean size from 4.0 to 53.0 mm. Despite a relatively long-term follow-up, no case had evidence of tumor recurrence. CONCLUSION: We evaluated the various anatomic locations of pathologically proven myofibroblastoma in Korean patients. As an extremely rare tumor, physicians should pay special attention to differential diagnosis. Surgical resection is the preferred method for a cure, and the recurrence rate is extremely low.

Tumor miofibroblástico de laringe: reporte de caso clínico y revisión de literatura / Miofibroflástico larynx tumor: case report and literature review

El tumor miofibroblástico inflamatorio de laringe es una patología rara dentro de los tumores de cabeza, cuello y de vía aérea, con una baja prevalencia, existiendo escasa literatura al respecto. Corresponde a un tumor benigno que generalmente se presenta como hallazgo incidental en relación a cuerdas vocales con mayor frecuencia, que puede ser de carácter asintomático o presentarse con clínica de obstrucción de vía aérea como estridor y disnea progresiva, además de disfonía. Puede afectar a pacientes de cualquier edad, siendo más frecuentes en adultos en edad media. El diagnóstico se hace a través de radiología y visualización directa mediante laringoscopía con la consiguiente obtención de muestra y estudio histopatológico. El tratamiento consta en la resección quirúrgica completa de la lesión, asociado o no a terapia esteroidal, sin embargo la curación completa no se logra en todos los casos producto de la naturaleza recidivante de la lesión.

The inflammatory myofibroblastic umor of the larynx is a rare condition in tumors of head, neck and airway, with a low prevalence, and scarce existing literature. It corresponds to a benign tumor that usually occurs as an incidental finding in relation to vocal cords more frequently, which can be asymptomatic or present with clinical character of airway obstruction, as progressive stridor and dyspnea, plus dysphonia. It can affect patients of any age are more common in adults in middle age. The diagnosis is made by radiology and direct laryngoscopy with visualization by obtaining sample and histology. Treatment consists of surgical removal of the entire lesion with or without steroid therapy, however complete healing is not achieved in all the cases product of the recurrent nature of the patology.

Mammary-Type Myofibroblastoma: A Report of Two Cases

Mammary-type myofibroblastoma (MFB) is a rare, benign spindle cell neoplasm occurring along the milkline, with extension from the mid-axilla to the medial groin. It is histologically and immunohistochemically identical to MFB of the breast and is part of a spectrum of lesions that includes spindle cell lipoma and cellular angiofibroma. Recently, we experienced two cases of mammary-type MFB involving male patients aged 30 and 58 years, respectively. The tumors were located in the right scrotal sac and in the right axilla. Wide excisions were performed. Microscopically, the masses were composed of haphazardly arranged, variably sized fascicles of bland spindle cells and were admixed with mature fat tissue. The spindle cells in both cases showed immunopositivity for desmin and CD34 and negativity for smooth muscle actin. Loss of retinoblastoma (RB)/13q14 loci is a characteristic genetic alteration of mammary-type MFB, and we identified loss of RB protein expression by immunohistochemical staining. We emphasize the importance of awareness of this rare neoplasm when a spindle cell neoplasm is accompanied by desmin immunopositivity. The second patient was alive without recurrence for 20 months, and the first patient had not been followed.

Granular cell tumor of rectal submucosa: case report / Tumor de células granulares da submucosa rectal: relato de caso

This is a case report of granular cell tumor of rectal submucosa in a female, 35-years-old patient complaining of hematochezia. We describe the histological and immunohistochemical features of the lesion responsible by this clinical find. Following that, we present a discussion of the case based on the literature review, which allowed to proving the infrequency of the tumor in the rectal area and confirms the benign nature of the tumor in this case. (AU)

Este artigo relata o caso de um tumor de células granulares da submocosa retal, em paciente de 35 anos, com queixa de hematoquezia. Fazemos a descrição dos achados histológicos e imuno-histoquímicos da lesão. Além disso, apresenta-se uma discussão do caso com base na revisão da literatura, que permitiu comprovar a infrequência do tumor na região retal e corroborar a benignidade do tumor no presente caso. (AU)

The Juscelino Kubitschek government and the Brazilian Malaria Control and Eradication Working Group: collaboration and conflicts in Brazilian and international health agenda, 1958-1961 / O governo JK e o Grupo de Trabalho de Controle e Erradicação da Malária no Brasil: encontros e desencontros nas agendas brasileira e internacional de saúde, 1958-1961

Malaria, a disease which was under control in the beginning of Juscelino Kubitschek government, became the most important endemic disease in 1958, when Brazil made a commitment with the World Health Organization to convert its control programs into eradication programs. For this purpose a Malaria Control and Eradication Group was set up under the leadership of the malaria specialist Mário Pinotti. Malaria would become an important bargaining chip in the context of the development policies of Kubitschek. This article focuses on path of the Malaria Control and Eradication Working Group in Brazil, in its varying relationships with the arguments and guidelines established at international level.

A malária, doença que estava controlada no início do governo de Juscelino Kubitschek, torna-se a mais importante endemia em 1958, quando o Brasil assumiu o compromisso com a Organização Mundial da Saúde de converter seus programas de controle em programas de erradicação. Para isso foi instalado um Grupo de Controle e Erradicação da Malária sob a direção do malariologista Mário Pinotti. A malária seria uma importante moeda de negociação no contexto da política de desenvolvimento de Kubitschek. Este artigo tem como foco a trajetória do Grupo de Trabalho de Controle e Erradicação da Malária no Brasil, em suas diferentes relações com as discussões e normativas travadas e estabelecidas em âmbito internacional.

Clinical analysis of imflammatory myofibroblastic tumor of the nasal cavity and paranasal sinus / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the clinical features, therapeutic methods and therapeutic efficacy of imflammatory myofibroblastic tumor(IMT)of the nasal cavity and paranasal sinuses.@*METHOD@#Clinical data of 14 cases diagnosed as IMT by pathology were retrospectively analyzed. There were 8 males and 6 females,age ranging from 18 to 77 years. 12 cases of them were treated by surgery while 2 cases received postoperative radiotherapy.@*RESULT@#All cases were operated. All the patients were followed up for a period of 1 to 7 years after operation and two cases were proved low grade IMT pathologically. Eight cases survived with no recurrence until the last follow-up and 6 cases relapsed, of which 4 cases died and 2 were alive with tumor.@*CONCLUSION@#IMT of the nasal cavity and paranasal sinuses is very rare. The diagonosis of IMT is based on pathology and immunohistochemistry. Proper diagnosis is essential to avoid mutilating and disfiguring surgical procedures. Radical excision is still the first choice of treatment for IMT of the nasal cavity and paranasal sinuses. Chemotherapy and radiotherapy may not be helpful to prevent recurrence after operation. Due to high recurrence rate, long-term follow up is necessary after operation.

A Case of Myofibroblastoma in the Submandibular Region / 대한이비인후과학회지

Myofibroblastoma of the mammary type is a benign breast mesenchymal tumor. It occurs commonly in the breast, but very rarely at extra mammary sites. The extra mammary myofibroblastoma is usually located on the embryonic milkline, which is extremely rare in the head and neck area. We report the case of a 14-year-old boy who was diagnosed with mammary type myofibroblastoma in the submandibular region. In this study, we focus on the clinicopathologic features of this unusual tumor along with the literature review.

Clinical analysis of 14 patients with extraorbital inflammatory myofibroblastic tumor of the head and neck / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To summarize the clinical features of extraorbital inflammatory myofibroblastic tumor (IMT) of the head and neck.</p><p><b>METHODS</b>Fourteen cases of extraorbital IMT treated in recent 20 years were analyzed retrospectively.</p><p><b>RESULTS</b>Of the 14 patients, 9 cases with limited lesion in maxilla (n = 5), mandible (n = 2) or neck (n = 2) underwent local resection, and no recurrences were found after 1.5 to 20.0 years; 3 cases diagnosed as maxillary IMT involved in orbit, hard palate or pterygopalatine fossa received conservative therapy (prednisone, prednisone plus radiotherapy or prednisone plus chemotherapy), and no disease progression was found after 6, 9 or 2 years respectively; and 1 case diagnosed as maxillary IMT involved in orbit and pterygopalatine fossa was confirmed with cervical metastases after two operations and died of brain invasion within 17 months. One patient with localized lesion around the common carotid artery was treated with prednisone and had no disease progression with a 2-year follow-up.</p><p><b>CONCLUSIONS</b>Extraorbital IMT of the head and neck is a rare clinical entity. Pathology examination is required for final diagnosis. Corticosteroid administration may be a choice of treatments, and radical resection should be taken selectively for limited lesions.</p>

Solid variant of angiomatoid fibrous histocytoma:report of 3 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotype, molecular genetics and differential diagnosis of solid variant of angiomatoid fibrous histocytoma.</p><p><b>METHODS</b>The clinicopathologic features of 3 cases of solid variant of angiomatoid fibrous histocytoma were analyzed and the literature was reviewed.</p><p><b>RESULTS</b>There were a total of 2 males and 1 female. The age of patients ranged from 9 to 12 years. The patients presented with a painless mass located in left forearm, left knee or back. The lesions were treated by complete surgical resection. On gross examination, the tumors varied from 1.6 cm to 4.5 cm in greatest dimension. They were well-circumscribed and had pale yellow to grayish-red solid cut surface. Histologically, the tumor was composed of histocytoid cells arranged in sheet-like pattern. A fibrous pseudocapsule surrounded by lymphocytes and plasma cells was identified. Immunohistochemical study showed that the tumor cells in all cases were positive for vimentin and CD68. They were negative for S100 protein, cytokeratin, CD34, CD31, smooth muscle actin, CD35, CD21 and CD30. Two cases also expressed CD99 and one of them was positive for desmin and epithelial membrane antigen. Fluorescence in-situ hybridization was positive for EWSR1 gene.</p><p><b>CONCLUSIONS</b>Solid type represents a variant of angiomatoid fibrous histocytoma and is considered as tumor of borderline malignant potential. Definitive diagnosis requires thorough histologic examination and clinical correlation. Immunohistochemistry and EWSR1 gene study are helpful in further delineation and differential diagnosis. Complete resection or wide local excision with post-operative follow up is the main modality of treatment.</p>

Tumores mesenquimáticos de vulva: reporte de tres casos / Mesenchymal tumors of the vulva: report of three cases

Los tumores mesenquimáticos benignos de vulva, denominados genéricamente como "tumores benignos del estroma genital", son lesiones raras, siendo las más frecuentes el pólipo fibroepitelial estromal, angiofibroma celular, angiomiofibroblastoma, angiomixoma superficial y leiomioma, los cuales comparten características morfológicas que dificultan su diagnóstico clínico y anatomopatológico, teniendo importancia su distinción, debido a las diferentes conductas terapéuticas a tomar en cada situación. Se presentan tres casos de pacientes que consultan por tumoración en labio mayor de vulva, con diagnóstico clínico de quiste de glándula de Bartholino, a quienes se les extirpa la lesión y realiza estudio anatomopatológico, resultando en diferentes tipos de lesiones sólidas de estirpe mesenquimática, por lo que se realiza revisión bibliográfica y análisis de diagnósticos diferenciales.

Extranodal Rosai-Dorfman disease of upper respiratory tract: a clinicopathologic study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of extranodal Rosai-Dorfman disease (RDD) of the upper respiratory tract.</p><p><b>METHODS</b>The clinical, pathologic and immunohistochemical features of 10 cases of RDD were evaluated.</p><p><b>RESULTS</b>Among the 10 cases studied, there were 3 males and 7 females. The age of patients ranged from 20 to 61 years old (mean 38 years). The lesion arose in the nasal cavity (7 cases), nasopharynx (2 cases) or hard palate to trachea (1 case). Most of the patients presented with nasal obstruction, rhinorrhagia or tumor mass in the nasal/nasopharyngeal regions. CT scan often showed the presence of soft tissue lesion without bone destruction. Histologically, extranodal RDD was characterized by light-staining bands alternating with dark-staining bands. The light-staining bands were formed by aggregates of large round or polygonal histiocytes with emperipoiesis. The dark-staining bands were formed by abundant lymphoplasmacytic infiltrates. Immunohistochemical study showed that the histiocytes strongly expressed S-100 protein and partially expressed CD68. Six patients had no recurrence after surgical resection.</p><p><b>CONCLUSIONS</b>Extranodal RDD of the upper respiratory tract is a rare disorder of histiocytic proliferation, which usually involves the nasal cavity and paranasal sinuses. RDD can easily mimic rhinoscleroma, mainly due to the overlapping morphologic appearance. Immunohistochemical study is helpful in the differential diagnosis.</p>