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PubMed2004

Role of chemotherapy in patients with soft tissue sarcomas.

The management of soft tissue sarcomas has been highlighted in the last few years by the responsiveness of gastrointestinal stromal tumors to imatinib (Gleevec, Novartis). In this article, the use of chemotherapeutic agents in the management of this and some of the 50 or more subtypes of sarcomas are discussed, and a brief review of the use of chemotherapy in the adjuvant or neoadjuvant setting for people with large extremity sarcomas is provided. Doxorubicin and ifosfamide (Mitoxana, Bristol-Myers Squibb) remain the best individual drugs for sarcomas overall, although dacarbazine and gemcitabine (Gemzar, Eli Lilly) with or without a taxane has activity in at least a subset of sarcomas. The data regarding adjuvant chemotherapy for extremity soft tissue sarcomas is still quite mixed, with little if any overall survival advantage found to support its incorporation into disease management. The finding of tyrosine kinase inhibitors such as imatinib with demonstrated activity in gastrointestinal stromal tumors and dermatofibrosarcoma protuberans, as well as the finding of new agents such as ecteinascidin-743 (Yondelis, PharmaMar) with at least some activity against soft tissue sarcomas, reinforces the idea that we should target individual subtypes of sarcoma, just as treatment varies by subtype for the hematological malignancies.

Chemotherapy, Adjuvant↗

PubMed1995

Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location.

OBJECTIVE: The purpose of this study was to determine the relative prevalence, age at presentation, sex distribution, and skeletal distribution of malignant soft-tissue tumors and to ascertain the relative frequency of these tumors in specific anatomic locations and age groups among a population of patients in a large pathologic consultation service. MATERIALS AND METHODS: The computer diagnoses of 39,179 lesions occurring in 38,484 patients seen by soft-tissue pathologists at the Armed Forces Institute of Pathology during the 10-year period from January 1, 1980, to December 31, 1989, were retrospectively reviewed. All lesions were placed in one of 121 major categories in accordance with the classification system used by the World Health Organization and coded to one of 32 anatomic locations, such as hand, wrist, forearm, and so forth. Age and sex also were recorded. For purposes of analysis, all lesions were placed in one of 10 categories: hand and wrist, upper extremity, proximal limb girdle (axilla and shoulder), foot and ankle, lower extremity, hip and buttocks region, head and neck, trunk, retroperitoneum, and other lesions. The study group included 31,047 mesenchymal lesions, of which 12,370 were malignant. RESULTS: More than 80% of malignant tumors were classified into eight diagnostic categories: malignant fibrous histiocytoma (24%), liposarcoma (14%), leiomyosarcoma (8%), malignant schwannoma (6%), dermatofibrosarcoma protuberans (6%), synovial sarcoma (5%), fibrosarcoma (5%), and sarcoma, not classified further (12%). Approximately 79% of all malignant tumors were classified into five diagnoses for each age and location. With the distal upper extremity (hand and wrist) as an example, 50% of malignant lesions in the 16-25-year-old group were classified as epithelioid sarcoma (29%), malignant fibrous histiocytoma (13%), and synovial sarcoma (8%). For the same location but for children 5 years old or younger, almost 50% of malignant tumors were classified as infantile fibrosarcoma. CONCLUSION: Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the location of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in establishing a suitably ordered differential diagnosis when a soft-tissue tumor has a nonspecific radiologic appearance.

Adolescent↗

PubMedDate not supplied

Comparative ultrastructure of soft-tissue myxoid tumors.

This comparative analysis of the characteristic ultrastructural features specific for intramuscular myxoma, myxoid liposarcoma, extraskeletal myxoid chondrosarcoma, myxoid malignant fibrous histiocytoma, myxoid variant of dermatofibrosarcoma protuberans, and myxoid neurilemmoma is based on our own studies of 52 cases. In spite of the histologic resemblance frequently observed, these tumors can be easily distinguished on the basis of cytoplasmic and extracellular features by electron microscopy. The value of electron microscopy in the diagnosis of myxoid tumors of soft tissues lies mainly in its potential to provide additional information concerning the cell types involved in the neoplastic process and their line of differentiation.

Chondrosarcoma↗

PubMedDate not supplied

Intracisternal paracrystalline serpigenous inclusions: a marker of disordered fibroblastic proliferation.

Three young children with characteristic fibroblastic tumors are presented. These comprised one case each of congenital fibrosarcoma, dermatofibrosarcoma protuberans, and fibrous hamartoma of infancy. Ultrastructural examination in each tumor revealed the presence of a peculiar intracisternal paracrystalline material that exhibited transverse periodicity between 18 and 22 nm. Elongated profiles lying within rough endoplasmic reticulum had a serpigenous outline. It is thought that this material is related to procollagen or its precursors and its presence may signify disorders of fibroblastic proliferation that may occur in fibrous tumors.

Cell Division↗

PubMed2000

Sarcomas of the head and neck at Kenyatta National Hospital.

OBJECTIVE: To determine the pattern of occurrence of sarcomas afflicting the neck and craniofacial region. DESIGN: A retrospective study (1982-1991). SETTING: Cancer Registry, Kenyatta National Hospital, Nairobi, Kenya. METHOD: Examination was performed of the cancer records in the registry over the period 1982 to 1991 inclusive. Histopathological types of the sarcomas were then analysed to indicate the numbers that occurred per year; and also the pattern of occurrence according to age groups, gender and site. RESULTS: Of the 10,897 whole body neoplasms, 985 were sarcomas. Among these sarcomas, 160 (16%) were located in the neck and craniofacial region. The histopathological types of the neoplasms included Kaposi's sarcoma (39%), osteosarcoma (23%), rhabdomyosarcoma (21%), fibrosarcoma (13%), chondrosarcoma (two per cent), malignant fibrous histiocytoma (one per cent) and dermatofibrosarcoma protuberans (one per cent). CONCLUSION: The results of this investigation, in concordance with those of other series worldwide, indicate that the various sarcomas afflicting the neck and craniofacial area are generally rare.

Adolescent↗

PubMed1998

[Cutaneous and subcutaneous soft tissue tumors].

Superficial, especially cutaneous soft tissue tumors deserve attention because of their special biology, often with better prognosis than their deep counterparts. This fact also has been considered in the TNM Classification/UICC, as well as in the Typing of Soft Tissue Tumors/WHO.-Basically all types of soft tissue tumors can also be found in subcutaneous and cutaneous localisation. Dermatofibrosarcoma protuberans, however, is a typical superficial tumor involving cutis and frequently the superficial subcutis. Superficial leiomyosarcoma and atypical fibroxanthoma are examples of sarcomas, which in spite of histological criteria, which generally are associated with high grade malignancy, behave as low grade malignant tumors, i.e. chiefly with risk of recurrency albeit negligible risk of metastases. Angiosarcoma and epitheloid sarcoma, on the other hand, may also metastasize, even if restricted to the cutis. Here histological criteria as numerous mitoses, cellular atypia and tumor necrosis, may additionally influence the prognosis. In conclusion it is evident that exact tumor classification is the basic requirement for an optimal, prognosis-adapted therapy. Prerequisite, however, is a precise clinico-pathological correlation to understand the biology of the different tumor entities.

Hemangiosarcoma↗

PubMed1998

[Cytogenetic changes in low grade central osteosarcomas].

Low grade central osteosarcomas are a group of rare bone neoplasms morphologically simulating benign bone tumors or tumor-like lesions (fibrous dysplasia, nonossifying fibroma, osteoblastoma, chondromyxoidfibroma or aneurysmal bone cyst). Because of the apparent bland histology they may be misinterpreted as benign lesions despite their primary low malignant behavior and late metastatic potential including the capability to transform into a highly malignant sarcoma. In contrast to high grade osteosarcomas cytogenetic studies of these entities are very rare. Karyotyping including FISH analysis with painting- and centromere-specific probes was done in 2 cases of low grade central osteosarcomas (fibrous dysplasia like type). One case was a primary lesion in the upper femur, the other was a recurrent lesion in the upper humerus. Additionally a static DNA cytometry using Feulgen stained imprints was carried out in both cases. In the first case clonal aberrations with a Robertsonian translocation between chromosomes #14 und #15, balanced translocations including chromosomes #5 and #21, #12 and #20, and a supernumary ring chromosome derived from chromosome #1 were detected in a near diploid karyotype. The second case displayed the development from a DNA-diploid to a DNA-peritetraploid lesion during a follow up of six years. Cytogenetic different marker chromosomes and again ring chromosomes were detected in a near tetraploid karyotype. Low grade central osteosarcomas of fibrous dysplasia like type may be characterized by chromosomal aberrations including ring chromosomes. Regarding the last feature these cytogenetic findings resemble those found in low grade parosteal osteosarcomas and other mesenchymal lesions of borderline behavior or low malignancy grade (for example dermatofibrosarcoma protuberans, low grade liposarcoma).

Adolescent↗

PubMed1998

[Fibrohistiocytic skin lesions].

AIMS: A new unifying concept of fibrohistiocytic skin lesions. METHODS: Retrospective clinicopathologic study of more than 2,000 dermatofibromas (DF) recruited over the last two decades. RESULTS: As the least common denominator all DF show a reactive fibrohistiocytic tissue response with variable epidermal hyperplasia, peripheral sclerosis and peripherally accentuated lymphohistiocytic tissue response. The histological, immunohistochemical and ultrastructural profile varies according to the time cycle as well as the fibro-myofibrohistiocytic differentiation of the lesions. From a conceptual point of view such lesions are best grouped into three categories: 1. DF with cellular/stromal pecularities. All classic variants, from the first description by Unna in 1894 to lesions described in 1978, namely DF, (benign) fibrous histiocytoma, histiocytoma cutis, sclerosing hemangioma, nodular subepidermal fibrosis or fibrous xanthoma, fall into this category; moreover, many of the clinicopathologic variants described over the last two decades such as granular cell, clear cell, myofibroblastic, sclerosing, monster cells, atypical ("pseudosarcomatous"), haemosiderotic ("elusive"), cholesterotic, and myxoid variants. 2. DF with architectural pecularities such as deep penetrating, atrophic, aneurysmal ("angiomatoid"), haemangiopericytoma-like, palisading and ossifying variants. And 3. DF with both cellular/stromal and architectural pecularities including those with a homogenous mixture of components as seen in epithelioid, cellular benign variants, smooth muscle proliferation in DF, multinucleate cell angiohistiocytoma, cellular neurothekeoma, and dermal plexiform fibrohistiocytic tumour; as well as those with heterogeneous mixture of components in composite or mixed dermatofibromas. CONCLUSION: DF is common, the clinicopathological variability manyfold, and their misinterpretation as malignancy such as dermatofibrosarcoma protuberans or Kaposi sarcoma not rare. Such cases have important clinical implications such as unnecessary investigations, controls and livelong anxiety of patients.

Histiocytoma, Benign Fibrous↗

PubMed1999

Occupational risk factors for sarcoma subtypes.

Herbicides, chlorophenols, and other occupational exposures are suspected risk factors for soft-tissue sarcoma, but the epidemiologic evidence is inconsistent. Given that soft-tissue sarcomas represent a heterogeneous mix of cancer subtypes and that these subtypes have different disease patterns by race, sex, and age at diagnosis, studying all soft-tissue sarcomas combined may mask subtype-specific associations. Using the Selected Cancers Study, a large population-based case-control study of sarcoma conducted among U.S. men aged 30 to 60 in 1984 to 1988, we explored the occupational risk factors for soft-tissue sarcoma subtypes and skeletal sarcoma. The analysis included 251 living sarcoma cases (48 dermatofibrosarcoma protuberans, 32 malignant fibrohistiocytic sarcoma, 67 leiomyosarcoma, 53 liposarcoma, and 51 skeletal sarcoma) and 1908 living controls. Exact conditional logistic regression models suggested patterns of subtype specificity for occupational exposures. Self-reported herbicide use was associated with malignant fibrohistiocytic sarcoma (OR = 2.9, 95% CI = 1.1-7.3). We found elevated risks for chlorophenol exposure and cutting oil exposure and malignant fibrohistiocytic sarcoma and leiomyosarcoma. We found no occupational risk factor for liposarcoma. Polytomous regression models identified different odds ratios across subtypes for plywood exposure and exposure to wood and saw dust. Although exploratory, this analysis suggests that occupational risk factors for sarcoma are not uniform across subtypes.

Adult↗

PubMed2000

[Tumoral mycetoma of the buttock].

BACKGROUND: Mycetomas are actinomycosic or fungal infections where the infectious agent produces grains. We report an atypical case of fungal mycetoma presenting as a tumoral formation on the buttock. CASE REPORT: A 56-year-old unemployed man from the Diourbel region of central Senegal consulted in February 1997 for a fistulalized tumor of the right buttock which had developed spontaneously and progressed for 5 years. The patient's general health remained satisfactory. Physical examination showed a voluminous 25 cm tumefaction extending from the right buttock to the perineum. The tumor showed a few areas of fistualization which discharged black grains under pressure. There was a 1.5 cm right inguinal node which did not appear to be inflammatory. The remainder of the physical examination was normal. Pathology reported inflammatory connective tissue surrounding a brownish polycyclic grain composed of spores and mycele filaments. The diagnosis of fungal mycetoma was retained and surgical excision under general anesthesia was performed. DISCUSSION: This is an atypical case of fungal mycetoma because of its tumoral form and gluteal localization. The differential diagnosis was cutaneous neurofibroma, myoma, or Darier-Ferrand dermatofibrosarcoma. The frequency of extrapodal red grain mycetomas has been pointed out by several authors from Senegal, but extrapodal black grain forms as seen in our case are more exceptional.

Buttocks↗

PubMed1976

Atypical fibroxanthoma of the skin. A clinico-pathological study of 57 cases.

A retrospective study of 57 patients with atypical fibroxanthoma of the skin is presented. The light microscopy is described and the differential diagnosis is discussed. Most of the atypical fibroxanthomas (32 out of 57 cases) were originally diagnosed as soft tissue sarcomas, e.g. fibrosarcoma, dermatofibrosarcoma, neurofibrosarcoma, myosarcoma and unspecified sarcoma. The tumour occurred chiefly in middle-aged and elderly patients; three patients were 15 years old or younger. The median age was 73 years in patients in whom the tumour occurred in the head and neck, and 34 years in patients in whom the tumour developed on the extremities and trunk. The sex ratio (male to female) was almost equal. Follow-up information about 43 patients was available. The follow-up period ranged from 1 year to 25 years with a median of 9 years. Eight patients died from intercurrent disease; all the other 35 patients are alive and well. The clinical course was benign in all but one patient in whom a recurrence developed and metastases to the regional lymph nodes appeared 7 years after the initial excision. The tumour in this case did not differ histologically from the other atypical fibroxanthomas with respect to cellularity, cellular and nuclear atypia or mitotic activity. It is suggested that the recurrence per se might be of prognostic importance.

Adolescent↗

PubMed2003

Skin cancers in Western Saudi Arabia.

OBJECTIVE: A retrospective analysis of skin cancers in a major referral centre in Taif region, Kingdom of Saudi Arabia, (KSA). METHODS: The case records of all malignant skin cancers diagnosed during a 10 year period, from 1992 through to 2001 were taken for the study. The clinical and histopathological details were noted. These were compared to reports from the rest of KSA and other countries. RESULTS: One hundred and four cases of malignant skin lesions including primary and metastatic tumors were seen. The majority were Saudis. The male to female ratio was 2.25:1. Most of the patients were over the age of 60 years. Basal cell carcinoma was the most frequent (51%) followed by squamous cell carcinoma (26%) and malignant melanoma (12.5%). Other rare primary tumors were those arising from the skin appendages, dermatofibrosarcoma protuberans and Kaposi's sarcoma. Metastatic skin lesions were seen in 5; in one it resulted from a surgical procedure and in the others the primary site could not be determined. CONCLUSION: The number of patients seen in this report is not high indicating that protective factors like clothing and skin type of the individual played a protective role. However, we feel that more studies should be encouraged in other regions along with the creation of a registry within each area to monitor the information regarding skin cancers. This could then be incorporated in health education programmes to be imparted to the public.

Adult↗

PubMed2004

A case of atypical benign fibrous histiocytoma.

A case of atypical benign fibrous histiocytoma is reported. A 62-year-old Japanese female visited our clinic because of an asymptomatic solitary lesion on the skin of the left leg. Physical examination revealed a polypoid mass lesion (2.5 x 2.3 x 1.8 cm) with central erosion. The lesion began with a 1 mm-sized papule and slowly enlarged over the 20 years. Clinical diagnosis was a malignant tumor such as dermatofibrosarcoma protuberans, atypical fibroxanthoma or adnexal tumors. Biopsy of the polypoid lesion was carried out. Histopathological examination revealed a polypoid lesion consisting of proliferation of fibroblast-like spindle cells in the dermis. Large atypical cells with pleomorphic nuclei were occasionally observed but mitotic figures were rare. From immunohistochemical results (CD68, Factor-XIII, MIB-1 labeling index), we diagnosed this case as "atypical benign fibrous histiocytoma (ABFH)". Clear distinction has not been made between ABFH, a variant of benign fibrous histiocytoma, and atypical fibroxanthoma, which is a variant of malignant fibrous histiocytoma. Here we report a case of ABFH with a diagnosis of the neoplasm.

Antigens, CD↗

PubMed2004

[Laparoscopic and general surgery guided by open interventional magnetic resonance].

Interventional magnetic resonance (IMR) machines have produced unique opportunity for image-guided surgery. The open configuration design and fast pulse sequence allow virtual real time intraoperative scanning to monitor the progress of a procedure, with new images produced every 1.5 sec. This may give greater appreciation of anatomy, especially deep to the 2-dimensional laparoscopic image, and hence increase safety, reduce procedure magnitude and increase confidence in tumour resection surgery. The aim of this paper was to investigate the feasibility of performing IMR-image-guided general surgery, especially in neoplastic and laparoscopic field, reporting a single center -- St. Mary's Hospital (London, UK) -- experience. Procedures were carried out in a Signa 0.5 T General Elettric SP10 Interventional MR (General Electric Medical Systems, Milwaukee, WI, USA) with magnet-compatible instruments (titanium alloy instruments, plastic retractors and ultrasonic driven scalpel) and under general anesthesia. There were performed 10 excision biopsies of palpable benign breast tumors (on female patients), 3 excisions of skin sarcoma (dermatofibrosarcoma protuberans), 1 right hemicolectomy and 2 laparoscopic cholecystectomies. The breast lesions were localized with pre- and postcontrast (intravenous gadolinium DPTA) sagittal and axial fast multiplanar spoiled gradient recalled conventional Signa sequences; preoperative real time fast gradient recalled sequences were also obtained using the flashpoint tracking device. During right hemicolectomy intraoperative single shot fast spin echo (SSFSE) and fast spoiled gradient recalled (FSPGR) imaging of right colon were performed after installation of 150 cc of water or 1% gadolinium solution, respectively, through a Foley catheter; imaging was also obtained in an attempt to identify mesenteric lymph nodes intraoperatively. Concerning laparoscopic procedures, magnetic devices (insufflator, light source) were positioned outside scan room, the tubing and light head being passed through penetration panels. Intraoperative MR-cholangiography was performed using fast spin echo (SSFSE) techniques with minimal intensity projection 3-dimensional reconstruction. About skin sarcomas, 2 of them were skin recurrences of previously surgically treated sarcomas (all of them received preoperative biopsy) and the extent of the lesion was then determined using short tau inversion recovery (STIR) sequence. The skin was closed in each case without need for any plastic reconstruction. The breast lesions were visualized with both Signa and real-time imaging and all enhanced with contrast: 2 (20%) were visualized only after contrast enhancement; intraoperative real time imaging clearly demonstrated a resection margin in all cases. Maximum dimensions of breast specimens (range 8-50 mm, median 24.5 mm) were not significantly different from those measured by Signa (p>0.17, Student's paired t-test) or real time images (p>0.4): also there was no significant difference in lesion size between Signa and real time images (p>0.25). All postprocedure scans clearly demonstrated complete excision. The extent of the tumor at MR imaging was greater in each case than suggested by clinical examination. Adequate resection margins were planned using STIR sequences. Histological examination confirmed clear surgical margins of at least 1 cm in each case. During right hemicolectomy, both intraoperative SSFSE and FSPGR contrast imaging revealed the lesion and details of the colonic surface; imaging of the lymph node draining right colon was only partially successful, due to movement artifact. Concerning laparoscopic procedures, both FSE and SSFSE techniques produced reasonable images of the gallbladder and intrahepatic ducts, but the FSE imaging was of poor quality due to respiration artifact; however, SSFSE allowed visualization of the gallbladder and part of the common bile duct. About skin sarcomas, the extent of the tumor at MR imaging was greater in each case than suggested by clinical examination and in each case the complete tumor excision was confirmed. Histological examination confirmed clear surgical margins of at least 1 cm in each case. Intraoperative MR scanning reliably identifies palpable breast tumours and skin sarcomas and is sufficiently accurate to guide their surgical excision. Further work may be done to develop laparoscopic and open abdominal surgery as well.

Anesthesia, General↗

PubMed2004

[Diagnostic significance and clinical application of specific chimeric genes in soft tissue sarcomas by RT-PCR using paraffin-embedded tissues: a study of 103 specimens].

OBJECTIVE: To investigate the expression of the chimeric genes resulting from the specific chromosomal translocations in soft tissue sarcomas (STS) and its diagnostic significance for STS. METHODS: The variety of fusion transcripts were detected in 103 cases of STS, including 30 cases of synovial sarcoma (SS), 15 cases of rhabdomyosarcoma (RMS), 25 cases of Ewing's sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET), 12 cases of dermatofibrosarcoma protuberans (DFSP), 14 cases of aveolar soft part sarcoma (ASPS), 3 cases of leiomyosarcoma (LMS), 2 cases of malignant fibrous histocytoma (MFH), and 2 cases of fibrosarcoma (FS); and 20 cases of control tumors by reverse transcription-polymerase chain reaction (RT-PCR) using formalin fixed, paraffin embedded specimens. RESULTS: Of the 34 cases of SS 28 (93.3%) expressed SSX-SYT chimeric transcripts (14 were positive for SYT-SSX1, 9 for SYT-SSX2). Four of the six cases of alveolar RMS had a PAX3/PAX7-FKHR fusion transcript. None of the 9 cases of embryonic and polymorphic RMS expressed PAX3/PAX7-FKHR. Of the 25 cases of ES/pPNET, 19 were positive for EWS-FLI1 fusion transcript and 1 for EWS-ERG fusion transcript. COL1A1-PDGFB fusion transcript was expressed in 8 of the 12 cases (66.7%) of DFSP. Of the fourteen cases of ASPS, ten expressed ASPL-TFE3 fusion transcript. None of the 3 cases of LMS, 2 cases of MFH, 2 cases of FS, and 20 control cases contained any of the fusion transcript. CONCLUSION: Chimeric gene transcript resulting from specific chromosomal translocations is a reliable index for the molecular diagnosis of STS and RT-PCR assay for detection of specific fusion gene provides a useful tool for confirmation of the diagnosis of STS in diagnostically difficult cases and in retrospective studies.

Artificial Gene Fusion↗

PubMed2004

[Imatinib--a new perspective in the treatment of tumors].

Among novel promising approaches to anticancer therapy belongs the targeting inhibition of signal transduction. This review outlines present-day experiences with imatinib (Glivec), a potent inhibitor of the tyrosine kinases bcr-abl, c-kit and platelet-derived growth factor receptor kinase. Due to inhibition of bcr-abl tyroxine kinase, imatinib has rapidly become the standard therapy for chronic myelocytic leukemia; inhibition of c-kit receptor explains its effectivity in the treatment of patients with gastrointestinal stromal tumors. Another known target of imatinib is tyrosine kinase of PDGFR, which is activated in numerous malignancies, particularly in dermatofibrosarcoma protuberans. Discovery of the novel fusion gene in hypereosinophilic syndrome (FIPILI-PFGFRA, whose product is an imatinib sensitive protein kinase) permitted to treat successfully this event. Possible combination of imatinib with conventional chemotherapeutic drugs and other key signal transduction inhibitors are mentioned.

Antineoplastic Agents↗

PubMed2005

[Cutaneous Rosai-Dorfman disease].

OBJECTIVE: To investigate the clinicopathologic feature, immunophenotype and differential diagnosis of cutaneous Rosai-Dorfman disease (CRDD). METHODS: Clinical manifestation, morphologic features and immunohistochemical staining were studied in 8 cases of CRDD. RESULTS: All 8 patients presented with multiple papules, nodules and/or coalescent patches or plaques distributing over the extremities or trunk, without lymphadenopathy or other systemic abnormalities. Microscopically, the lesions were located intradermally and/or subcutaneously. CRDD was characterized by the presence of S-100 positive histiocytic cells exhibiting emperipolesis, accompanying with infiltration of mixed inflammatory cells. Fibrosis, somewhere in vague storiform pattern due to stromal responses, with distribution of individual neutrophil microabscess was seen in cases with a long course of illness. Dilated vascular spaces in dermis containing numerous large typical histiocytes were seen in 2 cases. CONCLUSIONS: CRDD is a benign, persistent proliferative disease of histiocytes. Systemic involvement is rare, outcome favorable. It should be differentiated from other types of histiocytosis, dermatofibrosarcoma protuberans, xanthoma and lymphoproliferative disorders. Immunohistochemical staining for S-100 protein and CD68 is helpful in making a correct diagnosis.

Aged↗

PubMedDate not supplied

Microvessel density and HIF-1alpha expression correlate with malignant potential in fibrohistiocytic tumors.

Angiogenesis is a central process in the growth of solid tumors. Hypoxia-inducible factor-1alpha (HIF-1alpha) is an oxygen-dependent transcriptional activator, which plays a crucial role in tumor angiogenesis. However, involvement of HIF-1alpha has never been studied in so-called fibrohistiocytic tumors, such as dermatofibroma (DF), dermatofibrosarcoma protuberans (DFSP) and malignant fibrous histiocytoma (MFH). We analyzed the extents of angiogenesis in relation to the expression levels of HIF-1alpha in 26 DF, 13 DFSP and 23 MFH cases. MFH showed significantly higher microvessel density (MVD) compared with DF and DFSP. Immunohistochemically, HIF-1alpha-positive cases constituted 31%, 15% and 98% of DF, DFSP and MFH, respectively, indicating significantly higher HIF-1alpha expression in MFH compared with DF and DFSP. Furthermore, MFH cases expressing high levels of HIF-1alpha showed significantly higher MVD than those with low levels of HIF-1alpha. Thus, higher levels of angiogenesis and HIF-1alpha expression are both closely associated with the malignant potential in so-called fibrohistiocytic tumors, and HIF-1alpha is possibly involved in angiogenesis in MFH.

Adolescent↗