Kikuchi–Fujimoto disease: A rare cause of cervical lymphadenopathy

Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare and generally self-limiting disease of uncertain etiology that presents with fever and cervical lymphadenopathy in both the pediatric and adult populations. Here, we present the case of a 5-year-old male who presented with acute onset of fever and cervical lymphadenopathy which was initially diagnosed and managed as staphylococcal lymphadenitis. He underwent an excisional biopsy when symptoms persisted, which confirmed the diagnosis of KFD and was managed conservatively. This case study emphasizes awareness of this entity in the differential diagnosis of fever with persistent lymphadenopathy. Due to its characteristic overlap with other disorders such as tuberculous lymphadenitis, and lymphoma, KFD remains an arduous diagnosis for physicians. Therefore, awareness about its symptoms helps in minimizing potentially harmful unnecessary evaluations and thereby preventing misdiagnosis and inappropriate treatment.

Erdheim-Chester disease with multiple serous cavity effusion: a case report / 中国综合临床

Erdheim-Chester disease is a rare non-Langerhans histiocytic disease that can involve multiple systems, such as bone and joint, nerve, skin, large blood vessels and endocrine.The most common clinical manifestation is slight bone pain around the lower limb joints. The data reported a patient with multiple serous cavity effusion as the first manifestation and lack of symptoms of bone pain. PET-CT examination showed that the bone and large blood vessels were involved, and finally confirmed as Erdheim-Chester disease by bone biopsy pathology. The patient presented atypical manifestations of rare diseases, and the analysis of clinical data of the case will help improve the recognition of the disease by clinicians.

Kikuchi-Fujimoto disease. An unusual disease

Kikuchi-Fujimoto disease is self-limited, has a good prognosis, is diagnosed histopathologically, is more common on the Asian continent, in women and in those under the age of 40, and is usually diagnosed by exclusion. We present the case of a 20-year-old male patient with no significant medical history who consulted due to persistent fever, enlarged lymph nodes, gastrointestinal symptoms and weight loss. He debuted with pancytopenia, elevated acute phase reactants and altered liver function, and underwent an excisional lymph node biopsy, whose findings were compatible with Kikuchi-Fujimoto disease. He was treated symptomatically with NSAIDs and was followed up one month after discharge, at which time there was no evidence of recurrent fever or other additional symptoms. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2553).

La enfermedad de Kikuchi Fujimoto es autolimitada, de buen pronóstico, de diagnóstico histo-patológico, cuya incidencia es mayor en el continente asiático en mujeres, en menores de 40 años y usualmente es un diagnóstico de exclusión. Presentamos el caso de un paciente masculino de 20 años sin antecedentes patológicos, quien consultó por fiebre persistente, adenopatías, síntomas gastrointestinales y pérdida de peso; debutó con pancitopenia, aumento de reactantes de fase aguda, alteración de función hepática y en quien se le practicó biopsia excisional de ganglio, la cual evidenció hallazgos compatibles con la enfermedad de Kikuchi Fujimoto. Se hace manejo sintomático con AINES y se realiza control al mes de egreso, donde no hay evidencia de recurrencia de picos febriles u otros síntomas adicionales. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2553).

Kikuchi Fujimoto disease: a rare case report

Kikuchi disease, also known as Kikuchi histiocyticnecrotizing lymphadenitis, was initially described in young Japanese women. It is a rare benign self-limiting disease of unknown cause usually characterized by fever and cervical lunphadenitis. It is diagnosed by doing excisional biopsy of affected lymph node. A case of young 20 years old female presented with complaint of fever and weight loss for 20 days along with cervical and axillary lymphadenopathy. Complete physical examination, radiological investigations and biochemical tests were done to rule outsystemic lupus erythematous, non-Hodgkin lymphoma and tuberculosis.Only symptomatic treatment was done along with corticosteroids. Its diagnosis is important as it can be easily mistaken for other form of lymphadenitis. Clinician and pathologists’ awareness of this disorder is very necessary.

Hemophagocytic syndrome as the first presentation of histiocytic necrotizing lymphadenitis: report of 1 case and review of literature / 白血病·淋巴瘤

Objective:To investigate the diagnosis and treatment of histiocytic necrotizing lymphadenitis (HNL) complicated with hemophagocytic syndrome (HPS).Methods:The clinical characteristics, diagnosis, treatment process, and therapy response of a patient with HNL complicated with HPS admitted to the Institute of Hematology & Blood Diseases Hospital Chinese Academy of Medical Sciences in March 2019 were retrospectively analyzed, and the literature was reviewed.Results:This 17-year-old female patient had fever with bilateral cervical lymphadenopathy as the first presentation, accompanied by cough and expectoration. After admission, the disease progressed rapidly, and the serum ferritin increased progressively.The regimen of hormone and etoposide was used to control the disease condition. The bone marrow smear revealed atypical lymphocytes and hemophagocytic phenomenon, and the pathological features of HNL in lymph node biopsy were observed. This patient was finally diagnosed as HNL complicated with HPS. The patient's condition was stable at 3-month follow-up after discharge.Conclusions:The clinical manifestations of HNL patients complicated with HPS are similar to other hematologic malignant diseases, and application of multiple laboratory and pathological examination methods can help with early diagnosis. In the event of a progressive rise in serum ferritin, timely application of hormone therapy combined with etoposide if necessary can rapidly control the progression of the disease.

Disseminated mucocutaneous trichosporonosis in a patient with histiocytic sarcoma

Abstract Trichosporon asahii is the causal agent of trichosporonosis. Patients with immunosuppression or hematological malignancies are at higher risk of infection. Skin and mucosal involvement appear as fast-growing papulonodular lesions and necrotic ulcers. Internal organ dissemination is lethal. Therapeutic success depends on the underlying disease. Here, the authors present the first case of disseminated mucocutaneous trichosporonosis in a patient with a post-mortem diagnosis of histiocytic sarcoma, a rare and aggressive haematolymphoid neoplasm. Regretfully, death occurred despite treatment with liposomal amphotericin B and supportive measures, showcasing the fatality of both diseases.

Reto Diagnóstico: Hipoacusia, Enteropatía Autoinmune y Linfadenopatia de Kikuchi ­ un Debut Inusual de LES / Diagnostic challenge: hearing loss, autoimmune enteropathy and Kikuchi lymphadenopathy - An unusual debut of SLE

El lupus eritematoso sistémico (LES) puede presentarse de muchas maneras y puede ser difícil de diagnosticar. En ocasiones puede ser difícil distinguir la enfermedad subyacente, ya que se sabe que el LES mismo causa síntomas gastrointestinales e incluso malabsorción. Este caso refleja los desafíos de diagnóstico que enfrentamos durante la larga y complicada hospitalización de una adolescente con compromiso sistémico, síndrome febril, malabsorción, marcadores inflamatorios elevados, hipoacusia, nefritis, linfadenitis necrotizante histiocítica

Systemic lupus erythematosus (SLE) can present in many ways and can be difficult to diagnose. It can sometimes be difficult to distinguish the underlying disease, as SLE itself is known to cause gastrointestinal symptoms and even malabsorption. This case reflects the diagnostic challenges we faced during the long and complicated hospitalization of an adolescent with systemic compromise, febrile syndrome, malabsorption, elevated inflammatory markers, hearing loss, nephritis, histiocytic necrotizing lymphadenitis

Enfermedad de Kikuchi-Fujimoto / Kikuchi-Fujimoto disease

RESUMEN ntroducción: la enfermedad de Kikuchi-Fujimoto es una causa rara de linfadenopatía cervical. Se caracteriza por una proliferación focal de células reticulares acompañadas de numerosos histiocitos y restos nucleares extensos. Objetivo: presentar un paciente con enfermedad de Kikuchi-Fujimoto diagnosticado por el departamento de Anatomía Patológica. Presentación del caso: paciente masculino con VIH, color de la piel negra, 33 años de edad, acudió a consulta por presentar un síndrome febril prolongado y adenopatías en la región del cuello. Con la administración de anestesia local y sedación se realizó escisión de adenopatías y se diagnosticó una linfadenitis necrotizante histiocítica o enfermedad de Kikuchi-Fujimoto. Conclusiones: la enfermedad de Kikuchi-Fujimoto es una entidad rara y de etiología incierta. El diagnóstico certero se obtiene del estudio histopatológico de una biopsia y el tratamiento incluye el uso de antipiréticos y antiinflamatorios no esteroideos.

ABSTRACT Introduction: Kikuchi-Fujimoto disease is a rare cause of cervical lymphadenopathy. It is characterized by a focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear remains. Objective: to present a patient with Kikuchi-Fujimoto disease diagnosed by the Department of Pathology. Case report: a 33-year-old, male, black race, HIV patient came to the clinic for presenting a prolonged febrile syndrome and lymphadenopathy in the neck region. With the administration of local anesthesia and sedation, adenopathies excision was performed and a histiocytic-necrotizing lymphadenitis or Kikuchi-Fujimoto disease was diagnosed. Conclusions: Kikuchi-Fujimoto disease is a rare entity with an uncertain etiology. The accurate diagnosis is obtained from the pathologic-histological study of a biopsy and the treatment included the use of antipyretics and non-steroidal anti-inflammatory drugs.

Tumor Fibro-histiocítico Plexiforme de Vulva: Relato de Caso / Vulva Plexiform Fibrohistiocytic Tumor: Case Report / Tumor Fibrohistiocítico Plexiforme de la Vulva: Reporte de Caso

Introdução: O tumor fibro-histiocítico plexiforme é uma condição clínica rara, com cerca de 150 casos descritos na literatura. Relato do caso: Paciente, 23 anos, sexo feminino, portadora de lesão nodular em região vulvar sem características de malignidade em exames de ultrassonografia. Realizou acompanhamento clínico, tendo apresentado aumento do tamanho da lesão inicial, sendo optado então por realização de ressecção cirúrgica da lesão. Em estudo histopatológico, evidenciou-se lesão compatível com tumor fibro-histiocítico plexiforme, com margens cirúrgicas livres. No seguimento, a paciente foi encaminhada para avaliação oncológica com a intenção de realizar exames complementares de rastreio e descartar diagnósticos morfologicamente semelhantes. Diante dos exames complementares confirmando o diagnóstico inicial e sem evidência de doença metastática após excisão cirúrgica completa com margens livres, foi iniciado seguimento clínico. Conclusão: Relatar o caso é de extrema importância para divulgar a apresentação clínica, etapas diagnósticas, tratamento proposto e compartilhar informações acerca de evolução clínica apresentada.

Introduction: Plexiform fibrohistiocytic tumor is a rare clinical condition, with about 150 cases described in the literature. Case report: 23-years-old, female patient with nodular lesion in vulva without malignancy characteristics on ultrasound image. She underwent clinical follow-up and after an increase in the size of the lesion, surgical resection was opted. Histopathological findings suggested plexiform fibrohistiocytic tumor, with free surgical margins. In follow up, she was referred to oncological evaluation for screening complementary exams and rule out morphologically similar diagnoses. After the complementary exams confirming the initial diagnosis of plexiform fibrohistiocytic tumor and without evidence of metastatic disease after complete surgical excision with free margins, the patient is in clinical follow-up. Conclusion: Reporting the case is extremely important to disseminate the clinical presentation, diagnostic steps, proposed treatment and share information about the clinical evolution presented.

Introducción: El tumor fibrohistiocítico plexiforme es una condición clínica poco frecuente, con unos 150 casos descritos en la literatura. Reporte del caso: Paciente de 23, sexo feminino, años con lesión nodular en una región vulvar sin características malignas en los exámenes de ultrasonido. Se sometió a un seguimiento clínico, habiendo presentado un aumento en el tamaño de la lesión inicial, y se decidió realizar la resección quirúrgica de la lesión. En un estudio histopatológico, se evidenció una lesión compatible con tumor fibrohistiocítico plexiforme con márgenes quirúrgicos libres. Fue referida para la evaluación oncológica, realizó pruebas complementarias para el cribado y para descartar diagnósticos morfológicamente similares. En vista de las pruebas complementarias que confirman el diagnóstico inicial y sin evidencia de enfermedad metastásica después de una escisión quirúrgica completa con márgenes libres, se inició el seguimiento clínico. Conclusión: Informar del caso es extremadamente importante para difundir la presentación clínica, etapas diagnósticas, tratamiento propuesto y compartir información sobre la evolución clínica presentada.

Splenic histiocytic sarcoma: report of one case and review of literature / 白血病·淋巴瘤

Objective:To improve awareness of the diagnosis and treatment of splenic histiocytic sarcoma.Methods:The clinical data of one patient with splenic histiocytic sarcoma who was admitted to First Teaching Hospital of Tianjin University of Traditional Chinese Medicine in July 2020 were retrospectively analyzed, and the relevant domestic and foreign literature was reviewed.Results:The patient was diagnosed as splenic histiocytic sarcoma by histopathological and immunohistochemical examinations. The methylprednisolone combined with cyclosporine was ineffective. The patient received a total splenectomy, followed by chemotherapy with VECD and CHOP regimens. The patient's condition was stable during the 5-month follow-up after the operation. The result of bone puncture showed that there was no infiltration of histocytic sarcoma, and hematological remission was obtained.Conclusions:Splenic histocytic sarcoma is a highly malignant tumor with insidious onset, unclear pathogenesis, and lack of specificity in clinical manifestations and imaging examinations. The diagnosis depends on pathological biopsy and immunohistochemistry, and needs to be differentiated from other malignant tumors of lymphoid hematopoietic tissue. At present, there is no best treatment for splenic histiocytic sarcoma, and most patients have a poor prognosis.

A metastatic histiocytic sarcoma case with primary involvement of the tonsil

Histiocytic sarcoma (HS) is an extremely rare and aggressive hematopoietic tumor. Although it can be seen at any anatomic location, the most common primary sites are skin as extranodal region, locations including the lymph nodes and gastrointestinal tract. To the best of our knowledge, in light of PubMed search, this is the first primary tonsillar HS case presented with disseminated metastases at the time of diagnosis. A 58-year-old male patient applied with swelling on the right side of the neck, difficulty in swallowing, and weight loss. Positron emission tomography computed tomography was performed and increased pathological 18F fluorodeoxy D glucose uptake was detected in the right palatine tonsil, bilateral cervical multiple lymph nodes, liver masses, intra abdominal lymph nodes, and nodular lesion in the left adrenal gland. Tonsillectomy was performed and the pathological result was reported as HS. The patient did not respond to any treatment and had died after 5 months from the date of diagnosis. In conclusion, HS is generally diagnosed at advanced stage, it has limited chemotherapy response and high mortality rates. To understand this rare disease's pathophysiological and clinical features, further investigations are needed

Adenomegalia dolorosa: doença de Kikuchi entre os diagnósticos diferenciais / Painful adenomegaly: Kikuchi disease among differential diagnoses

RESUMO: A doença de Kikuchi-Fujimoto ou linfadenite histiocítica necrosante, de curso benigno, é prevalente em mulheres jovens, e associada à febre e leucopenia. Estudos recentes têm demostrado que sua etiologia ainda é incerta, sendo uma doença rara, com incidência de 0,5 a 5% de todas as adenopatias analisadas histologicamente. O diag-nóstico diferencial por imunohistoquímica foi decisivo, descartando outras hipóteses diagnósticas como: linfoma, tuberculose ganglionar e lúpus eritematoso sistêmico. Este relato de caso mostra as características da apresentação da doença em uma mulher, caucasiana e todas as etapas da investigação, destacando a importância dos diagnósticos diferenciais em adenopatias dolorosas e as dificuldades quando avaliamos portadores de doenças raras. (AU)

ABSTRACT: Kikuchi-Fujimoto disease or benign necrotizing histiocytic lymphadenitis is prevalent in young women and associated with fever and leukopenia. Recent studies have shown that its etiology is still uncertain, being a rare disease, with an incidence of 0.5 to 5% of all histologically analyzed adenopathies. Differential diagnosis by immunohistochemistry was decisive, ruling out other diagnostic hypotheses such as lymphoma, ganglion tuberculosis, and systemic lupus erythematosus. This case report shows the characteristics of the presentation of the disease in a Caucasian woman and all stages of the investigation, highlighting the importance of differential diagnoses in painful adenopathies and the difficulties when evaluating rare-disease patients. (AU)

Pediatric multifocal histiocytic sarcoma- a fatal diagnosis not to miss!

Histiocytic sarcoma (HS) is a rare hematolymphoid malignant neoplasm with an aggressive clinical course. It can arise de novo or from low-grade B-cell lymphoma. We describe the case of a 16-year-old boy referred to our hospital with generalized lymphadenopathy, weight loss, and decreased appetite for one month. The patient died undiagnosed on the 7th day of hospitalization. Lymph node and bone marrow biopsies were performed one day before the patient died. The lymph node biopsy revealed an architectural effacement with a diffuse proliferation of large pleomorphic neoplastic cells containing large, multilobulated nuclei, coarse vesicular chromatin, prominent nucleoli, and a moderate amount of eosinophilic cytoplasm. The bone marrow aspiration smears and biopsy also showed evidence of infiltration by these above-mentioned cells. Based on the morphology, along with the exclusion of many differential diagnoses by an extensive panel of immunohistochemical markers, a diagnosis of HS was made. This case report aims at evaluating all the clinical and immunophenotypic features of a case of HS with multifocal presentation and an aggressive clinical course in order to give a correct and definite diagnosis at the proper time.

Chronic histiocytic intervillositis in consecutive miscarriages: A potential pitfall in routine examination of conceptus

@#Introduction: Chronic histiocytic intervillositis (CHI) is a rare placental lesion strongly associated with recurrent miscarriages and fetal losses. It requires histopathological diagnosis and can only be made after delivery of the products of conception (POC). We describe a case of CHI in a 41-yearold lady with a 16-year history of thirteen recurrent consecutive first trimester miscarriages. Case report: The patient is a 41-year-old lady who suffered first trimester miscarriages in all her thirteen pregnancies. The relevant clinical investigations revealed neither significant nor helpful findings in determining the cause of recurrent miscarriages. Histological findings in each except one of the submitted conceptual tissue showed similar features of histiocytic aggregates primarily within the intervillous spaces, a characteristic description of CHI. One of the samples showed degenerative changes. Discussion: Practicing pathologists are not familiar with the histological features of CHI and this may be a potential pitfall in routine examination of POCs. Recognising this entity allows for accurate diagnosis and hence better management. The aetiology remains unclear, although an immunopathological basis are being explored.

Benign Cephalic Histiocytosis: A Rare Dermatological Entity in the Paediatric Population

@#Histiocytic proliferation of the skin can be categorised into Langerhan and non-Langerhan types. Benign cephalic histiocytosis (BCH) is a rare cutaneous non-Langerhan histiocytosis typically affecting infants and young children. We report a case of benign cephalic histiocytosis in a boy who, at seven months of age, presented with multiple yellowish papular eruptions on his face. Over the course of 18 months, the lesion spread to his trunk and upper extremities, sparing the mucous membranes, palms and soles. There was no systemic involvement identified. A histopathologic examination of the skin lesion showed diffuse infiltration of histiocytes within the superficial dermis intermingled with scattered eosinophils and small lymphocytes. Immunohistochemical studies showed that the histiocytes were diffusely positive for CD68. Langerhan markers CD1a and S100 were negative. The correct distinction between BCH and other histiocytic proliferations of the skin is important because BCH has a self-limiting clinical course with a tendency of spontaneous remission.

The correlations between traditional Chinese medicine syndromes of histiocytic necrotizing lymphadenitis and its pathological types / 国际中医中药杂志

Objective@#To investigate the interaction between traditional Chinese medicine (TCM) syndromes of histiocytic necrotizing lymphadenitis (HNL) and pathological types of HNL, so as to discover the distribution rule of TCM syndromes for indiction of intervention of HNL.@*Methods@#A total of 166 patients with HNL were included. The baseline data of the patients, clinical feature, the four diagnosis information of TCM and histopathological of cervical lymph node were collected. The syndromes of TCM were judged. The pathological types were confirmed by cervical lymph node biopsy. The Multi-way ANOVA was used to analyze the correlation between traditional Chinese medicine syndromes of HNL and its pathological types.@*Results@#Among the 166 patients, the ratio of different types are as follow: wind-heat and phlegm-toxin syndrome (57.83%, 96/166), heat-toxin exuberance syndrome (34.34%, 57/166), and phlegm and blood stasis syndrome (7.83%, 13/166). The pathological types include proliferative type (57.83%, 96/166), necrotizing type (35.54%, 59/166), and xanthomatous type (6.63%, 11/166). There were significant differences in targeted lymph node diameter, pain scores, body temperature (F value were 3.737, 34.484, 34.805, all Ps<0.05), while the difference of WBC (F=1.194, P>0.05) among three TCM Syndrome types were notsignificant. There were significant differences in pain scores, body temperature (F value were 12.153, 25.931, all Ps<0.05), but not for the targeted lymph node diameter, WBC (F value were 2.249, 0.671, all Ps>0.05) among three pathological types. The corresponding analysis result showed that, on point sets, wind-heat and phlegm-toxin syndrome were correlated with proliferative type, heat-toxin exuberance syndrome correlated with necrotizing type, and phlegm and blood stasis syndrome correlated with xanthomatous type (χ2=235.100, P<0.001).@*Conclusions@#There is a close correlation between syndrome types of TCM and pathological types of HNL. The pathological change of wind-heat and phlegm-toxin syndrometends toproliferative type, heat-toxin exuberance syndrome tends to necrotizing type, and phlegm and blood stasis syndrome tends to xanthomatous type.

Efficacy of medicinal penetration on acupoint as adjunctive therapy on histiocytic necrotizing lymphadenitis of heat-toxin syndrome: a randomized controlled trial / 中国针灸

OBJECTIVE@#To evaluate the clinical efficacy of medicinal penetration on acupoint combined with medication for histiocytic necrotizing lymphadenitis (HNL) of heat-toxin syndrome, and to explore the methods of improving the clinical effect.@*METHODS@#A total of 72 cases with HNL with heat-toxin syndrome were randomly divided into an observation group and a control group, 36 cases in each group. The patients in the control group were treated with oral administration of prednisone tablets for 40 days (first 5 days: 10 mg, three times a day; since then, reduced by 5 mg every 7 days). In the observation group, on the basis of the medication in the control group, the patients were treated with acupoint application and ultrasonic drug penetration therapy, once a day for 14 days. The acupoints of Waiguan (TE 5), Fengchi (GB 20) of affected side and points were selected. The changes of target lymph node swelling, visual analogue score (VAS), axillary temperature and total score of symptoms and signs were evaluated before treatment and 7, 14, 28 and 40 d into treatment; the changes of white blood cell (WBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and lactic dehydrogenase (LDH) were evaluated on 14 d and 40 d into treatment; the patients were followed-up for half a year.@*RESULTS@#① Fourteen days into treatment, the clinical cured rate in the observation group was 38.9% (14/36), which was superior to 16.7% (6/36) in the control group (0.05). ④ The recurrence rate in the observation group was 5.6% (2/36), which was similar to 16.7% (6/36) in the control group (>0.05).@*CONCLUSION@#The medicinal penetration on acupoint as adjunctive treatment could effectively relieve the discomfort symptoms of HNL patients with syndrome of heat and toxin, improve the clinical cured rate, and provide the research direction for shortening the course of medication.

Estudio Observacional: Dermatofibrosarcoma Protuberans, Serie de casos / Observational Study: Dermatofibrosarcoma Protuberans, Case Series

Introducción: El dermatofibrosarcoma protuberans(DFSP), es uno de los tumores cutáneos menos frecuentes, se clasifica dentro de los sarcomas fibrohistociticos y está caracterizado por un crecimiento lento y progresivo. Métodos: Se realizó una serie de casos retrospectiva de los pacientes con DFSP atendidos en el Centro de la Piel (CEPI) entre 2010 y 2019, se tomó en cuenta la información demográfica, características clínicas, tratamiento y su evolución. Resultados: Seis pacientes fueron diagnosticados y tratados por DFSP en el período evaluado, existió un predominio de mujeres (4 mujeres, 2 hombres) con una media de edad de 58.2 (DE =19,6), tres de ellos presentaron lesiones en forma de placa indurada y los restantes presentaron nódulo único, la localización de las lesiones fueron en hombro, cuello e hipogastrio. El tratamiento más utilizado fue la escisión amplia, ninguno de los pacientes presentó recidivas al primer y quinto año del seguimiento. Conclusión: Es necesario el reconocimiento de las características clínicas típicas de DFSP ya que podría pasar como una tumoración benigna de piel, por lo cual se debe apoyar con el estudio histopatológico frente a la sospecha de este tipo de lesión

Introduction: Dermatofibrosarcoma protuberans (DFSP) is one of the rare tumors found in the fibrohistocytic sarcomas and it is characterized by slow and progressive growth. Methods:A retrospective study of six cases treated at the Skin Center (CEPI) between 2010 and 2019 was carried out. We have take into account demographic information, characteristics, treatment and evolution of the lesions. Results:Six patients were diagnosed and treated by DFSP. There was a predominance of women (4 women, 2 men) with a mean age of 58.2 (SD = 19.6), three of them presented lesions in the form of indurated plaque and the rest presented a unique nodule. The locations of the lesions were the shoulder, neck and hypogastrium. The most commonly used treatment was wide excision, none of the patients presented recurrences at the first and fifth year of follow-up. Conclusion:Recognition of the typical clinical characteristics of DFSP is necessary since it could be mistaken as a benign tumor of the skin. The diagnosis must be always done by histopathological confirmation

Histiocytic Sarcoma Of The Small Intestine: A Case Report With Review Of The Literature

Histiocytic sarcoma is very rare malignant neoplasm showing morphological and immunophenotypic characteristics of histiocyte. Morphologic diagnosis is challenging due to overlapping histologic features with diverse mimics. Histologic diagnosis is confirmed by expression of one or more histiocytic markers, including CD163, CD68, and lysozyme. We present a bona fida case of extranodal histiocytic sarcoma involving the small intestine in a 67-year-old male. The diagnosis is confirmed by diffuse immunoreactivity of CD163, which is believed to be more specific histiocytic marker than CD68.

Extranodal histiocytic sarcoma mimicking colorectal lymphoma: Case report and review of literature

Histiocytic sarcoma is a rare malignant neoplasm that demonstrates mature histiocytic traits as characterized by immunohistochemistry. We report a case of extranodal histiocytic sarcoma (ENHS) of colon in a 56-year-old man presenting with gastrointestinal symptoms. Radiological findings were indicative of lymphoma or diffuse metastatic disease in colon. Histopathology of colectomy specimen was suggestive of ENHS, and immunohistochemical studies confirmed the uncommon diagnosis. The patient refused further therapy and succumbed to systemic complications of metastatic disease within a month of diagnosis. There have only been seven previous reports in world literature of ENHS involving large intestine.