A case of gastric crystal-storing histiocytosis.
Yoshiaki IsonoYouichirou BabaHiroki TanakaHiroaki KumazawaTomomasa TochioShinpei MatsuzakiTomohiro SaseTomonori SaitoHiroshi OkanoKatsumi MukaiRiuko Ohashi
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Abstract:
A 54-year-old male patient underwent upper gastrointestinal endoscopy, which revealed a 25-mm brown region in the angular section of the greater curvature of the stomach. The region was histologically determined to be gastric mucosa with an accumulation of histiocytes containing eosinophilic substances in the cytoplasm and chronic inflammatory cell infiltration. Histiocytes were immunohistologically positive for CD68, IgG, and κ. Based on these findings, the patient was diagnosed with gastric crystal-storing histiocytosis comprised of histiocytes phagocytosing IgG-κ-type immunoglobulin. This is a rare disease of which there have been no previous reports that included long-term follow-up. Here, we report the case with a literature review.Keywords:
CD68
Infiltration (HVAC)
A 54-year-old male patient underwent upper gastrointestinal endoscopy, which revealed a 25-mm brown region in the angular section of the greater curvature of the stomach. The region was histologically determined to be gastric mucosa with an accumulation of histiocytes containing eosinophilic substances in the cytoplasm and chronic inflammatory cell infiltration. Histiocytes were immunohistologically positive for CD68, IgG, and κ. Based on these findings, the patient was diagnosed with gastric crystal-storing histiocytosis comprised of histiocytes phagocytosing IgG-κ-type immunoglobulin. This is a rare disease of which there have been no previous reports that included long-term follow-up. Here, we report the case with a literature review.
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We first report a case of granular cell histiocytosis occurring as a solitary polypoid lesion of the nipple in a 15-year-old girl. Histologically, the lesion was composed of a dermal population of medium- to large-sized, short spindle- to round- to epithelioid-shaped cells with eosinophilic cytoplasm containing numerous and small diastase-resistant periodic acid-Schiff (PAS) positive granules. No associated inflammatory cells were observed. Immunohistochemical studies, revealing immunoreactivity exclusively to vimentin and CD68, were consistent with their histiocytic profile. Based on clinical, morphological and immunohistochemical features, the diagnosis of 'solitary cutaneous histiocytosis with granular cell changes' was proposed. The absence of an inflammatory cell component, such as lymphocytes and leucocytes, along with no history of a previous trauma or medical treatment, suggest that the present lesion could fit into the morphological spectrum of the so-called solitary epithelioid histiocytoma, also known as reticulohistiocytoma. Alternatively, the possibility of a histiocytic reaction to unknown stimuli cannot be completely ruled out. Nevertheless, awareness of solitary cutaneous histiocytosis with granular cell changes is useful to avoid confusion with other dermal tumors, especially 'granular cell tumor' and 'dermal non-neural granular cell tumor'.
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Massive accumulation of crystals within histiocytes in association with a lymphoplasmacytic neoplasm causing histologic features closely mimicking adult rhabdomyoma is a rare occurrence. We report the case of a 49-year-old man who presented with a left posterior cervical mass. Histologic examination demonstrated sheets of large, elongated, and polygonal cells with prominent eosinophilic cytoplasm surrounding scattered dense lymphoplasmacytic collections. Evidence of the reactive histiocytic nature of the eosinophilic cells was provided by an immunohistochemical reaction that was positive with KP-1 (CD68), but negative with common muscle actin, desmin, and myoglobin. The ultrastructural finding of elongated and rhomboid, membrane-bound, cytoplasmic crystals further supported the reactive histiocytic nature of these cells. B-cell kappa monoclonality of the lymphoplasmacytic component was proven by gene rearrangement studies. A recurrence involving the right parotid gland, 18 months after removal of the cervical mass, confirmed the aggressive nature of this B-cell lymphoma. Misdiagnosis of crystal-storing histiocytosis as adult rhabdomyoma can be avoided if the following features are recognized: lack of cytoplasmic cross striations, frequent multinucleation of the histiocytes, and prominence of the atypical lymphoplasmacytic component.
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The paper describes a case of eosinophilic granuloma of the parietal bone in a 32-year-old man. Histological examination revealed a large number of bean-shaped Langerhans cell histiocytes with lobed nuclei and nuclear grooves. The histiocytes alternated with the foci of obvious eosinophilic infiltration and with eosinophilic microabscesses. There were osteoclast-like multinucleated giant cells, bone resorption, and numerous bone rods covered with osteoblast chains. The histiocytes expressed CD1α, langerin, CD68, S100, and p53 (in 90.0% of the tumor cells). The Ki-67 proliferation index was 18.0%. A molecular genetic study identified BRAFV600E mutation (nucleotide substitution s.1799 T>A (p.V600E) in the heterozygous state). Clinical and morphological data and the results of molecular genetic studies led to the conclusion that there was eosinophilic granuloma of the right parietal bone (the unifocal form of Langerhans cell histiocytosis (LCH), type I, group A1, with the monoossal nature of lesion and with BRAFV600E mutation). In adults, this disease is extremely rare (2-5 cases of LCH per million people, bone loss in the fourth decade of life in 2.5% of the patients).Приведено наблюдение эозинофильной гранулемы теменной кости у мужчины 32 лет. При гистологическом исследовании определялось большое количество гистиоцитов Лангерганса бобовидной формы с дольчатыми ядрами и ядерными бороздками. Гистиоциты чередовались с фокусами выраженной эозинофильно-клеточной инфильтрации и эозинофильными микроабсцессами. Определялись остеокластоподобные многоядерные гигантские клетки, резорбция костной ткани, многочисленные костные балки, покрытые цепочками остеобластов. Гистиоциты экспрессировали CD1α, лангерин, CD68, S100, р53 (в 90% клеток опухоли). Индекс пролиферации Ki-67 составил 18%. При молекулярно-генетическом исследовании выявлена мутация гена BRAFV600E (нуклеотидная замена с.1799 Т>A (p.V600E) в гетерозиготном состоянии). На основании клинических и морфологических данных, а также результатов молекулярно-генетического исследования сделано заключение об эозинофильной гранулеме правой теменной кости (юнифокальная форма лангергансоклеточного гистиоцитоза (ЛКГ), тип I, группа А1, с монооссальным характером поражения и мутацией гена BRAFV600E). У взрослых данное заболевание встречается крайне редко (2-5 случаев ЛКГ на 1 млн человек, поражение костной ткани на 4-м десятилетии жизни у 2,5% больных).
Eosinophilic granuloma
Langerhans Cell Histiocytosis
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Langerin
Parietal bone
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Background: Multicentric reticulohistiocytosis (MRH) is a rare histiocytic proliferative disorder involving skin, mucosa and joints. Diagnosis is confirmed by histopathological examination. Case Report: A 45 year old patient presented with non-pruritic papules over the body and multiple joint pains. Histopathologically, the lesion was composed of oncocytic macrophages and multinucleate giant cells with abundant, eosinophilic and granular cytoplasm with ground glass appearance. Immunohistochemical expression for CD-68 and S-100 was seen . Conclusion: We describe a case of MRH along with brief review of literature with unusual immunohistochemical expression of S-100 protein which is reported negative in majority of previously presented cases, however CD68 positivity confirmed the non-langerhans cell origin.
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The objective of this study was to explore the diagnosis and therapy of extranodal Rosai-Dorfman disease(RDD).Two cases of extranodal RDD were studied by the hematoxylineosin and immunohistochemical staining,along with literature review.The lesions of RDD were characterized by the presence of large histiocytes with emperipolesis,accompanied by infiltration of lymphocytes,plasma cells and other inflammatory cells.The large histiocytes had an abundant cytoplasm,pale to eosinophilic in appearance,positive for S-100 and CD68 protein staining.The different therapies resulted in different prognosis.In conclusion,extranodal RDD is known as an idiopathic proliferative disease of histiocytes with a distinct morphologic feature and is very rare.It is difficult to diagnosis because of its multiple appearance.The efficacy of therapy and prognosis depend on its appearance.
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We describe the case of a 37-year-old male patient who in 1992 started developing a skin eruption characterized by dark red to red-yellow papulonodular lesions that showed no tendency to spontaneous resolution. Visceral organs were not involved and the lipid pattern was basically normal. Histological examination revealed an infiltrate in the mid and upper dermis mainly consisting of mononucleated and multinucleated histiocytes with an abundant eosinophilic cytoplasm and a ground glass appearance, admixed with numerous xanthomatized cells and Touton and foreign-body giant cells. Immunohistochemical studies showed positivity for CD68 and negativity for CD1a and S-100 protein, whereas at the electron microscopy level the only peculiar finding was the presence of many desmosome-like junctions. The authors believe this to be a borderline form between papular xanthoma and reticulohistiocytosis.
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Multinucleate
Langerhans Cell Histiocytosis
Birbeck granules
Xanthoma
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To study the clinicopathological features, diagnosis and prognosis of primary sinus histiocytosis (Rosai-Dorfman disease, RDD) of the trachea by case report and review of the literature.A 63 year old man with a space-occupying lesion of the trachea firstly diagnosed as a malignant tumor was admitted to this hospital for further evaluation and treatment. The lesion was removed by surgery and the final diagnosis was primary RDD. The clinical data of the case was analyzed and the related literatures were reviewed. The literature review was carried out respectively with"Rosai-Dorfman disease" and "sinus histiocytosis"as the key words in Wanfang Med Online and with"Rosai-Dorfman disease","sinus histiocytosis","trachea or lung"as the key words in PubMed database by July 2012.The chest computerized tomography of the case showed that the mass was located at the right side of the trachea with heterogeneous density and contrast enhancement. Bronchoscopy revealed a neoplasma occluding the distal trachea. The lesion was excised by surgery. Microscopic histology showed that in the dark-staining area a large number of lymphocytes and plasma cells were noted while the light-staining area was formed by giant histiocytes. The pathological changes invaded the tracheal wall and eroded the cartilages. Intact lymphocytes and plasma cells were observed within the eosinophilic cytoplasm of the histiocytes. Immunohistochemistry showed that the giant histiocytes were strongly positive for S-100 protein and CD68 protein. Primary RDD of trachea was confirmed. The patient remained well without any other treatment or evidence of progression for 11 months. A total of 13 literatures and 26 cases were retrieved from Wanfang Med Online and Pubmed, including 21 cases of primary RDD of the upper respiratory tract and 4 cases of primary RDD of the lung. A total of 5 literatures and 5 cases of RDD affecting the trachea were retrieved from Wanfang Med Online and Pubmed. There was only one case of primary RDD of the trachea in Pubmed. A 39-year-old female patient with 1 month of dyspnea was misdiagnosed as having bronchial asthma and was unresponsive to empirical corticosteroid and bronchodilator therapy. The chest computerized tomography revealed an ill-defined irregular soft tissue in the trachea. A tracheal ring sleeve resection and reanastomosis was performed to prevent asphyxia. The mass was confirmed to be primary RDD of the trachea according to histopathology and immunohistochemistry. The patient was well without any treatment for 12 months.Primary RDD of the trachea is an extremely rare disease, with dyspnoea as a feature of the disease. When it is completely removed, the prognosis is good. Typical histopathology and immunohistochemistry are needed to make a definite diagnosis. The positive immunohistochemistry staining for S-100 and CD68 protein in giant histiocytes and lymphocyteemperipolesis are essential for the diagnosis. The differential diagnosis includes other benign or malignant space-occupying lesions of the trachea.
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Malignant histiocytosis
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Generalized eruptive histiocytoma (GEH) is a very rare benign disorder belonging to the group of non-Langerhans cell histiocytosis (non-LCH). GEH is characterized by a nearly uniform infiltrate of histiocytes with classic immunological phenotype (CD68+, S-100- and CD1a-). Prominent eosinophilic infiltration and S100-positive histiocytes are rarely associated in GEH. In this article, we reported a middle-age man presented with disseminated reddish papules distributed on the trunk and proximal extremities. A skin biopsy of the papule showed a dense histiocytic infiltration with prominent eosinophils. By immunohistochemistry, the histiocytes revealed strongly positive for CD68 and S100 protein and negative for CD1a and Langerin (CD207). Based on clinical and histopathological criteria, the diagnosis of GEH was established. We presented this rare case of GEH with such distinctive features to strengthen the awareness of this uncommon form of non-LCH. Classical histopathological and immunological features cannot reliably distinguish GEH from other non-LCH.
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S100 protein
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