Intravascular fasciitis of the forearm vein: A case report with immunohistochemical characterization

Intravascular fasciitis is a very unusual variant of nodular fasciitis. A unique case of this lesion occurring in the proximal portion of the superficial vein of the forearm in an otherwise healthy 26-year-old man is reported. The intravascular polypoid lesion grew longitudinally along the vascular lumen, was loosely attached to the intimal layer, and was partly anchored beyond the internal elastic lamina into the medial smooth muscle layer. However, extravascular involvement was absent. The histological features were identical to those observed in ordinary cellular nodular fasciitis. Because of its myofibroblastic phenotype exhibited by highly proliferative spindle cells, certain intimomedial myofibroblasts are thought to be the indigenous source of this unique fibroproliferative lesion. Unless the diagnosis of intravascular fasciitis is considered and appropriate differential markers examined, it may be confused with other intravascular lesions, such as intravascular leiomyoma, intravenous pyogenic granuloma, organized thrombus and, even, fibromuscular dysplasia if it arises in the arteries. A simple excision is considered curable. Even so, two recurrent cases have been documented to date.     

Intravascular fasciitis of the hip joint in a postpartum female: misdiagnosed as low grade fibromyxoid sarcoma

Intravascular fasciitis is a special type of nodular fasciitis. It is an uncommon lesion. We report here the first case of intravascular fasciitis involving the deep muscle of the hip joint. The female, postpartum patient presented with a large, firm, painless tumoral mass in the anterolateral muscles of the right hip. The diagnosis of intravascular fasciitis was difficult because of the large size and location of the lesion. The false positive immunohistochemistry for MUC4 initially caused our team to misdiagnose the intravascular fasciitis as a low grade malignant fibromyxoid sarcoma. Our case adds to the literature on intravascular fasciitis. Since the operation about 20 months prior, the tumor in this case has not recurred.     

Nodular fasciitis of the hand: a potential diagnostic pitfall in fine-needle aspiration cytopathology

Fine-needle aspiration biopsy (FNAB) is applied very uncommonly to soft tissue masses and even more infrequently to lesions of the hand. Nodular fasciitis, an uncommon pseudosarcomatous lesion of soft tissue, rarely occurs in the hand and, because of this, is not often considered in the differential diagnosis of hand masses. We report 3 cases (2 men and 1 woman; mean age, 44.3 years) of soft tissue masses of the hand, which, after clinical and radiologic evaluation, were strongly suspected by an experienced orthopedic oncologist as harboring a soft tissue sarcoma. Each patient underwent FNAB, which showed markedly hypercellular smears with overlapping, relatively isomorphic spindle cells that were mistaken cytologically as possible sarcoma in 2 cases; 1 case was considered probable nodular fasciitis. All lesions eventually were diagnosed as nodular fasciitis after thorough histologic and immunohistologic evaluation. Nodular fasciitis remains a difficult diagnosis by FNAB, particularly when it occurs in locations such as the hand.     

增生性筋膜炎临床病理分析

目的：探讨增生性筋膜炎的临床病理特点。方法：例1为男性患者,74岁,发现右颌下区包块1周;例2为女性患者,71岁,发现左颈部包块1周,迅速长大3 d。观察2例增生性筋膜炎的组织学及免疫表型并复习相关文献。结果：2例患者镜下见肿块由大量形态不规则的梭形细胞组成,呈致密区与疏松区交替排列。致密区中梭形细胞排列成编织状、席纹状。疏松区梭形细胞呈束带状排列于胶原和黏液样间质为主的间质中,其间可见神经节样大细胞。免疫组织化学显示：例1梭形细胞的波形蛋白（vimentin）、平滑肌肌动蛋白（smooth muscle actin,SMA）、钙调蛋白结合蛋白和肌动蛋白结合蛋白呈阳性表达,不表达可溶蛋白-100（soluble protein-100,S-100）和细胞分化簇（cluster of differenation 34,CD34）;例2梭形细胞的vimentin和SMA呈阳性表达,不表达细胞角蛋白（cytokeratin,CK）、S-100、人黑色素相关抗体（human melanoma-related antibody 45,HMB45）和CD34,两例Ki-67阳性率均低于2%。结论：增生性筋膜炎是一种罕见的良性增生性病变,手术切除是主要治疗手段。因其临床及病理特征,易被误诊为恶性肿瘤而予以后续的放射治疗和化学治疗,故应加强对该病的认识,避免误诊和漏诊。     

Fasciitis ossificans of the breast

We report a rare case of fasciitis ossificans of the breast. A nodule of the breast was incidentally found in a 77-year-old woman without a history of trauma. The painless lesion was located 3 cm from the nipple in the lower outer quadrant of the left breast. Excisional biopsy was performed, and there has been no evidence of recurrence or metastasis for 2 years. The 1.8 cm diameter nodule was well demarcated from the adjacent tissue and located 2.5 cm beneath the skin. Histologically, the lesion was composed of uniform woven bone trabeculae with rimming of osteoblasts and fibrous stroma. At the periphery, spindle cells actively proliferated in edematous stroma, demonstrating uniform nuclei without increased chromatin, pleomorphism, or evident nucleoli. We counted 2 mitotic figures per 10 high-power fields, but no atypical forms were observed. Spindle cells were immunoreactive for vimentin and alpha-smooth muscle actin, suggesting myofibroblastic differentiation. Fasciitis ossificans is histologically identical to myositis ossificans, but tends to present no zonation phenomenon. We considered this lesion as fasciitis ossificans since it was situated at the superficial layer of the mammary gland. To avoid an unnecessarily aggressive treatment, fasciitis ossificans, a benign bone-forming nodule, needs to be considered in the differential diagnosis of breast hard tumor.     

Parosteal fasciitis of the clavicle

A rare case of parosteal fasciitis arising from the periosteum of the left clavicle in a 27-year-old woman is reported. Magnetic resonance imaging demonstrated the lesion surrounding the periosteum of the clavicle. The lesion was iso-intense with muscle on T(1)-weighted images and hyperintense on T(2)-weighted images. At surgery, the lesion was discovered to be densely adherent with the periosteum, and excised along with the periosteum. Histopathological examination revealed the proliferation of myofibroblasts in a vague storiform or short fascicular pattern. A large amount of extravasated erythrocytes, and a few lymphocytes were present in the matrix. There were some foci of abundant myxoid materials. Immunohistochemical study showed the cells to be positive for vimentin, alpha-smooth muscle actin and HHF35, but negative for desmin. There was no local recurrence at a 6 months postoperative follow up.     

Intraarticular nodular fasciitis causing limitation of knee extension: A case report

We report a patient with intraarticular nodular fasciitis who developed mechanical symptoms in the knee, limiting knee extension. A 24 year-old man presented with mechanical restriction of extension and pain at maximum extension. MRI revealed an intraarticular mass within the knee joint. The mass was excised arthroscopically. Histological examination of the excised tissue led to a diagnosis of nodular fasciitis. Pain and limitation of motion of the knee disappeared from one day after surgery. No recurrence was found after 1 year. Intraarticular nodular fasciitis is rare, and although this lesion within the knee has been reported previously, it has not been associated with mechanical symptoms. This case indicates that intra-articular fasciitis should be considered in the differential diagnosis of a patient presenting with mechanical limitation of knee extension and a mass lesion inside the knee joint.     

Fibro-osseous pseudotumor of digits - Expanding the spectrum of clonal transient neoplasms harboring USP6 rearrangement

Fibro-osseous pseudotumors of the digits (FOPD) is a rare self-limiting lesion composed of bland looking hypercellular fibrous tissue and bone.USP6 rearrangement is a consistent genetic finding in aneurysmal bone cyst, nodular fasciitis, myositis ossificans and giant cell lesions of small bones.We report herein the occurrence of USP6 rearrangement in fibro-osseous pseudotumors of the digits using fluorescence in situ hybridization analysis (FISH).Of the five patients included, three were female and two were male. The age ranged from 33 to 72 years (mean 48 years). Lesions arose in the palm (n = 2), thenar (n = 1), middle finger (n = 1) and great toe (n = 1). All patients underwent resection.Four cases (80%) harbored USP6 rearrangements showing that fibro-osseous pseudotumors of digits belongs to the spectrum of clonal transient neoplasms including aneurysmal bone cyst, nodular fasciitis, myositis ossificans and giant cell lesion of small bones.     

Pseudosarcomatous lesions of the soft tissues reported as sarcoma during a 6-year period (1958-1963)

Pseudosarcomatous lesion of the soft tissues is a term used in the present study for various soft tissue lesions and tumours easily clinically or histologically, or both, misinterpreted as sarcoma. Eighty-one cases, that is to say 10 per cent of all tumours classified and reported to the Swedish Cancer Registry as malignant soft tissue tumours during the 6-year period studied (1958-1963), were reclassified as pseudosarcomatous lesions of the soft tissues. Forty-seven cases were classified as pseudoarcomatous proliferative lesions of the soft tissue with or without bone formation; 38 cases of nodular fasciitis, 1 of proliferative fasciitis and 8 of proliferative myositis. In 3 of these cases there were mixed forms of proliferative fasciitis and proliferative myositis with areas compatible with the diagnosis of nodular fasciitis evident in all cases. Twenty-two cases of atypical fibroxanthomas of the skin were next in frequency, followed by 7 ancient neurilemmomas, 2 spindle cell lipomas, 1 pseudomalignant osseous tumour of the soft tissues, 1 pigmented villonodular synovitis and 1 juvenile xanthogranuloma. An attempt is made to explain the reasons for these erroneous diagnoses of sarcoma and it is stressed that for these lesions the conventional histological criteria for malignancy are not valid. The awareness and knowledge of the existence of these particular entities are therefore considered mandatory for an accurate diagnosis.     

Cytological findings in nodular fasciitis

Nodular fasciitis, proliferative fasciitis, and proliferative myositis are tumorous proliferative soft tissue changes which on histological examination are quite often erroneously diagnosed as malignant neoplasms. Reported are cytological and histological findings recorded from nodular fasciitis in a man aged 34 years. The cytomorphology of nodular fasciitis is sufficiently characteristic, so that the lesion can be differentiated with certainty from malignant processes, since the cells have no features of malignancy, and the admixture of granulocytes and histiocytes in smears suggests a proliferative inflammatory process.     

Articular nodular fasciitis of the right shoulder joint: report of an unusual case with focus on immunohistochemical differential diagnosis

The mesenchymal lesion nodular fasciitis (NF) can affect various sites of the body but usually arises in subcutaneous tissue or occasionally skeletal muscle. NF is not commonly known to arise in joints, and articular NF is extremely rare. Herein, we present a case of a 54-year-old woman with articular NF. No sign of recurrence was observed after surgical piecemeal removal with a suspected positive surgical margin. In our case, a differential diagnosis of NF, desmoid-type fibromatosis, and low-grade myofibroblastic sarcoma was considered. Stromal hyalinization, a characteristic of articular NF, made the diagnosis somewhat difficult, although typical NF morphology was present. Immunohistochemical analysis of α-smooth muscle actin, desmin, β-catenin, and protein gene product 9.5 expression along with close morphological examination provided a reliable distinction.     

Multiple metachronus proliferative fasciitis occurring in different anatomic regions: a case report and review of the literature

Proliferative fasciitis is a benign lesion that usually has a self-limited course and rarely recurs after excision. In the literature, the multifocal occurrence of PF in different anatomic sites has not been reported so far. In this report, we describe the clinical case of a 30-year-old woman with two metachronous proliferative fasciitis occurring firstly in the orbit and, after 18 months, in the forearm; we also review the available literature on this topic, outlining guidelines for therapy and the follow-up of these patients.     

Fine-needle aspiration cytology of angiolymphoid hyperplasia with eosinophilia: a case report with electron microscopy and immunohistochemistry

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon vascular inflammatory lesion usually involving the dermis or subcutaneous tissue of the head-neck region of middle-aged women. Histologically, this lesion shows a florid proliferation of vessels lined by particular endothelial cells and an inflammatory infiltrate composed of lymphocytes and eosinophils. The fine-needle aspiration (FNA) cytology of a nodular lesion in the retroauricolar region of a 18-yr-old woman showed spindle-shaped and polygonal cells with vesicular nuclei and deeply eosinophilic cytoplasm containing well-defined vacuoles in a background of eosinophils and lymphocytes. These features were consistent with a proliferation of epithelioid endothelial cells, and a diagnosis of ALHE was suggested. The histology confirmed the preoperatory diagnosis, and ultrastructural and immunohistochemical studies further demonstrated the endothelial nature of epithelial-appearing cells. Because the clinical appearance of the lesion may mimic a large number of benign and malignant diseases, a preoperatory diagnosis of ALHE is rarely made. The FNA cytology may represent a simple and reliable method with which to study and diagnose proliferations of epithelioid endothelial cells.     

Molecular characterization of methicillin-resistant Staphylococcus aureus from a fatal case of necrotizing fasciitis in an extremely low-birth-weight infant

Necrotizing fasciitis due to methicillin-resistant Staphylococcus aureus (MRSA) is an uncommon but life-threatening infection, and has mainly been reported as occurring in adults and the elderly. Recently, infant cases involving Panton–Valentine leukocidin (PVL)-positive community-acquired MRSA have been noted. Here, a case of fatal necrotizing fasciitis with sepsis and disseminated intravascular coagulation in an extremely low-birthweight infant is described. The causative agent was the hospital-acquired MRSA New York/Japan clone carrying the spa variant gene and nine staphylococcal enterotoxin (SE) genes. These data suggest that a high-level combination of SEs and other virulence factors, but not PVL, could contribute to the pathogenesis of fatal necrotizing fasciitis.     

Nodular fasciitis of the vulva: a challenging histopathologic diagnosis supported by the detection of USP6 gene rearrangement

Nodular fasciitis involving the vulva on physical examination can mimic a Bartholin gland lesion, and histologically can have overlapping features with more ominous mesenchymal pathologies. We describe a case in which a 52‐year‐old perimenopausal woman presents with a vulvar mass. After an initial biopsy and later excision, the myofibroblastic lesion was noted to have areas of differing cellularity, with compact nodule formations at the periphery. Immunohistochemical staining showed lesional cells to be positive for desmin, estrogen receptor, progesterone receptor, and smooth muscle actin, and negative for CD34, ALK‐1, myogenin, caldesmon, S100, and wide spectrum (Oscar) cytokeratin. Desmin is generally negative in this lesion type, but the positivity in this case was considered to be secondary to the origin of the myofibroblastic cells of the vulva. The morphologic pattern and immunophenotype favored a diagnosis of nodular fasciitis, however, the degree of hypercellularity and desmin positivity warranted further analysis. The diagnosis was supported with fluorescence in situ hybridization that demonstrated USP6 gene rearrangement. This highlights the necessity in certain challenging cases for ancillary molecular and/or cytogenetic analysis.     

Nodular fasciitis of the male breast: a case report

A case of nodular fasciitis is reported that involved the breast parenchyma of a 40-year-old man. The differential diagnosis of nodular fasciitis in the male breast mainly includes fibromatosis and myofibroblastoma. However, other benign and malignant spindle cell lesions of the breast, such as pseudoangiomatous stromal hyperplasia and especially spindle cell metaplastic carcinoma and fibrosarcoma, may enter the differential. The classic histomorphologic features and immunohistochemical findings are helpful in arriving at the correct diagnosis. The patient underwent a total excision of the lesion and is free of disease after 14 months. To our knowledge, this is the first reported case of a lesion of this type in the male breast.     

Subscapular elastofibroma intermingled with adipose tissue: variant type of elastofibroma or lipoma?

Elastofibroma is a rare entity most commonly seen in subscapular region. Elastofibrolipoma was first described by De Nictolis et al (Am J Surg Pathol. 1995;19:364-367) as a capsulated mass, composed of mature fat, abnormal elastic fibers, and connective tissue, at the anterior mediastinum. A 57-year-old woman with a periscapular pain and a mass at the left subscapular region was admitted at the department of thoracic surgery. Histologic sections of the well-circumscribed 6.5 x 5 x 3.5-cm rubbery mass revealed a collagenous stroma containing eosinophilic globules and fragmented fibers intermixed with mature adipose tissue surrounded by a thin fibrous capsule. The elastic nature of these eosinophilic fibers and globules was confirmed with Verhoeff's elastic stain. The lesion was diagnosed as elastofibrolipoma, a recently recognized lesion with an unclear origin. Here we discuss if it is a variant of elastofibroma or lipoma.     

Vimentin,smooth muscle actin,desmin, S-100 protein,p53, and estrogen receptor expression in elastofibroma and nodular fasciitis

Elastofibroma and nodular fasciitis are two rare, benign soft tissue tumors. Elastofibroma is suggested to develop as a result of abnormal degeneration of elastic fibers after local trauma. Similarly, fibroblastic proliferation, which is triggered by local trauma or nonspecific inflammatory event, is suggested to play an important role in the origin of nodular fasciitis. In this immunohistochemical study, vimentin, smooth muscle actin (SMA), desmin, S-100 protein, p53, and estrogen receptors were applied to paraffin sections of 10 elastofibroma and four nodular fasciitis specimens to learn more about their histogenesis and biological behavior. All cases with nodular fibrosis were strongly SMA and vimentin positive, while only three weakly stained with estrogen receptor antibody. There was no immunreactivity for S-100, desmin, and p53 in nodular fasciitis. However, all elastofibroma cases were stained positively with vimentin. No staining was observed with SMA, S-100, desmin, and p53 in elastofibroma. The staining pattern of nodular fasciitis supported a myofibroblastic origin, whereas the SMA negativity in elastofibroma was correlated with fibroblastic origin.     

Fibroma of tendon sheath.

A series of nine cases of fibroma of tendon sheath is described including details of the ultrastructural features of two cases. The series was composed of lesions from six males and three females with a mean age of 38 yr. The most common site of involvement was the hand (including fingers) and the mean greater diameter was 19 mm. Typically the tumours were lobulated and microscopically there was a collagenous stroma with spindle and stellate cells in a moderate degree of cellularity. One recurrence was noted in the series. The lesion was distinguished from circumscribed fibromatosis, nodular fasciitis, neurofibroma, leiomyoma, scar tissue, giant cell tumour of tendon sheath (localised nodular tenosynovitis) and fibrous histiocytoma. Ultrastructural studies revealed that the large majority of cells present in the two cases studied were myofibroblasts and fibroma of tendon sheath is therefore the third instance of a benign tumour containing these cells (the other two being dermatofibroma and giant cell fibroma of the oral mucosa).     

Nodular mucinosis of the breast: a case report with pathologic, ultrasonographic, and clinical findings and review of the literature

Nodular mucinosis is an extremely rare breast lesion. This benign mass usually presents clinically as a poorly circumscribed, subareolar, myxoid mass in young female patients. We report a case of this rare breast lesion and discuss its clinical, radiologic, and histopathologic features. A 21-year-old white woman presented with a mass in the left breast of 6 months' duration. She had never been pregnant or had any history of breast feeding, surgery, trauma, or use of exogenous hormones or a family history of breast cancer. Clinical breast examination demonstrated a 1 cm "rubbery" mass directly under and continuous with the left nipple. The skin that covered the mass had an edematous and irregular appearance without erythema or drainage from the nipple. Ultrasonography demonstrated a 1-cm, nonintraductal, circumscribed, homogeneous, isoechoic mass that was continuous or part of the base of the left nipple. The mass was smooth, with a thin echogenic rim. Doppler flow showed some vascularity. These findings suggested a benign breast lesion, including a fibroadenoma or nipple adenoma. Despite reassurance, the patient desired excision of the lesion. Gross examination revealed a nodular, rubbery-firm, ovoid, pink, polypoid mass that measured 1.5 x 0.9 x 0.8 cm. Microscopic examination showed a well-circumscribed tumor with a nodular appearance, which consisted of an accumulation of pink myxoid tissue and contained spindle cells with bland-appearing nuclei, no mitosis, and mild cellularity. The pink myxoid tissue was stained with Hale colloidal iron and Alcian blue. The Alcian blue stain was removed by pretreatment with hyaluronidase. The spindle cells stained with vimentin and smooth muscle actin; however, they did not express smooth muscle myosin or cytokeratin. This report presents and discusses the pathologic, ultrasonographic, and clinical findings of this rare entity.