Histiocytic sarcoma with fatal duodenal ulcers

Akishima, Yuri; Akasaka, Yoshikiyo; Ger, Yih-Chang; Ito, Kinji; Ishikawa, Yukio; Zhang, Lijun; Kiguchi, Hideko; Lipscomb, Gary; Strong, Jack P.; Ishii, Toshiharu

doi:10.1016/j.prp.2004.03.010

Cited by 15 publications

(9 citation statements)

References 20 publications

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“…General remarks and clinical presentation: Histiocytic sarcoma (HS) may affect all age groups, but has its highest incidence in the 5th decade of life. The majority of patients present with tumor bulks, mostly in lymph nodes, but also in extralymphatic organs, such as the skin [68,208] and the gastrointestinal tract [7,80,94,186,234]. Less often, primary lung disease resembling pulmonary LCH [196], and primary parotid gland involvement have been described [6].…”

Section: Histiocytic Sarcoma (Histiocytic Mh)mentioning

confidence: 99%

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The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

2016

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Rare histiocytoses, also called non-Langerhans cell histiocytoses, include all proliferative disorders of histiocytes, macrophages and dendritic cells that are not classified as Langerhans cell histiocytosis (LCH) and do not belong to the hemophagocytic lymphohistiocytosis (HLH) group of diseases. Thus, the term includes numerous benign or malignant, localized or systemic, adult or pediatric diseases. The classification of the histiocytic disorders has been revised several times. Here, we follow the classification recently published by Jean Francois Emile and an international expert panel, defining subgroups of histiocytoses described as L-Group, C-Group, M-Group, R-Group, and H-Group, which stands for LCH-like, cutaneous or mucocutaneous, malignant, Rosai-Dorfman-Disease like and HLH like. Some of the diseases have an excellent prognosis after resection or even disappear spontanously, others progress rapidly, requiring intensive systemic therapies. The malignant non-Langerhans cell histiocytoses in general have a poor prognosis, here, complex chemotherapy protocols are usually applied, with inconsistant results. An interesting perspective in non-malignant rare histiocytoses might be small molecular inhibitors, in particular BRAF inhibitors, since BRAF mutations have been found in some subtypes of non-Langerhans cell histiocytoses. By prospective and retrospective collection of experiences in a new registry (the "International Rare Histiocytic Disorders Registry", IRHDR), knowledge about these rare diseases might hopefully be improved.

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Section: Histiocytic Sarcoma (Histiocytic Mh)mentioning

confidence: 99%

“…Also, leukemia-like spread of cells in the blood may occur [36]. Unspecific symptoms such as fever, weight loss, hepatosplenomegaly and pancytopenia are observed as well [2,7,96,161,194]. Typically, the disease has an aggressive course, with poor prognosis [80,182,194].…”

Section: Histiocytic Sarcoma (Histiocytic Mh)mentioning

confidence: 99%

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

2016

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“…More than 57 cases, including 16 cases affecting the GI tract, have been reported between 2001 and 2010. Eight of the 16 cases of GI HS occurred within the small intestine . All cases were ultimately diagnosed via immunohistochemical studies, although the diagnoses were based on both morphological features and the presence of immunophenotypic features of histiocytic lineage (i.e., abundant eosinophilic cytoplasm, round to oval or irregularly folded nuclei and sometimes prominent nucleoli).…”

Section: Discussionmentioning

confidence: 99%

“…Eight of the 16 cases of GI HS occurred within the small intestine. [4][5][6][7] All cases were ultimately diagnosed via immunohistochemical studies, although the diagnoses were based on both morphological features and the presence of immunophenotypic features of histiocytic lineage (i.e., abun-dant eosinophilic cytoplasm, round to oval or irregularly folded nuclei and sometimes prominent nucleoli). Immunohistochemically, these neoplastic cells were positive for one or more histiocytic markers, such as CD163, CD68 and lysozyme and negative for markers of B and T cells, myeloid cells and epithelioid cells.…”

Section: Discussionmentioning

confidence: 99%

The utility of balloon enteroscopy in the diagnosis of histiocytic sarcoma of the small intestine: A case report

Miyazaki

Arihiro

Mitobe

et al. 2013

Pathology International

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The diagnosis of the rare neoplasm histiocytic sarcoma (HS) relies on morphology and the presence of immunophenotypic features of histiocytic lineage. More than 57 cases, including 16 cases involving the gastrointestinal (GI) tract, have been described since the World Health Organization issued its classification system for tumors of hematopoietic and lymphoid tissue in 2001. HS is often diagnosed in its late stages, at which point the prognosis is poor. Only a small proportion of these patients can undergo surgical resection with curative intent. The present report describes how HS can be diagnosed at a stage of favorable prognosis using balloon enteroscopy (BE), thereby enabling surgical resection before the development of metastases. This strategy is reviewed in the setting of a patient with jejunal HS, followed by a discussion of data from 16 other reported cases of GI HS.

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“…Histiocytic sarcoma in small bowel commonly manifests as polypoid tumors causing intestinal obstruction [ 5 , 6 ]. Multiple ulcerations, some with fatal perforation, were found in the esophagus and duodenum of a patient with histiocytic sarcoma [ 7 ]. They showed only non-specific inflammatory changes without tumor cell involvement.…”

Section: Discussionmentioning

confidence: 99%

Histiocytic Sarcoma of the Terminal Ileum Presenting As a Large Ulcerating Lesion: CT Diagnosis

Khan¹,

Agarwal²,

Chaddha³

et al. 2009

Radiology Case Reports

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We report the case of an 84-year-old woman with histiocytic sarcoma arising in the terminal ileum. The lesion was identified on CT of the abdomen as a 10 cm segment of terminal ileum with mild circumferential wall thickening, isoattenuation, and diffuse homogeneous enhancement. The patient was treated with surgery and adjuvant chemotherapy. This case provides an example of when imaging may be useful for the detection of this rare malignancy and its complications.

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Histiocytic sarcoma with fatal duodenal ulcers

Cited by 15 publications

References 20 publications

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

The utility of balloon enteroscopy in the diagnosis of histiocytic sarcoma of the small intestine: A case report

Histiocytic Sarcoma of the Terminal Ileum Presenting As a Large Ulcerating Lesion: CT Diagnosis

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