Cases reported "Fibroma"

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11/91. Fibromyxoma of the middle ear (a case report).

    Fibromyxoma of the middle ear (a case report). We report an extremely rare case of fibromyxoma of the middle ear which was first suspected to be a cholesteatoma. Surgery was performed through a postauricular incision and pathologic study of the tumor gave us the final diagnosis. Fibromyxoma is an uncommon benign tumour of the head and neck region which usually arises in the jaw (1). Its local aggressivity and gelatinous consistency explain the difficulties to remove it radically and its frequent recurrences after treatment (2). Surgical resection with a wide excision seems to be the treatment of choice. Some authors advocate the use of argon or CO2 laser to achieve the treatment. A review of the poor literature about this subject is discussed in this article.
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12/91. Intra-articular elastofibroma of the shoulder joint.

    A 19-year-old man presented with an elastofibroma in his left shoulder joint. The patient had had limitation of motion in his left arm for 3 years, especially when rotating internally. radiography of his left shoulder showed bone erosion in the neck of the humerus. On MR imaging, a soft tissue mass was noted in the shoulder joint eroding bone. The mass showed similar signal intensity to that of surrounding muscles on T1- and T2-weighted images. At surgery, a soft, encapsulated mass was found attached to the subscapularis muscle. It was pathologically confirmed as an elastofibroma. This unusual manifestation of an elastofibroma is discussed.
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13/91. Perifollicular fibromatosis cutis with polyps of the colon--a cutaneo-intestinal syndrome sui generis.

    In the present study, a peculiar fibromatosis cutis in two siblings has been reported the dermatosis being characterized by innumerable perifollicular fibromas on face, neck and trunk as well as multiple fibromata pendulantia. Since the father allegedly had skin lesions resembling those of his two affected children, an inherited condition is assumed for the disease manifesting itself rather late in age. In the female patient, several adenomatous colon polyps one being transformed into carcinoma were found. Since the association of distinct epithelial and mesenchymal tumours of the skin and the cranial bones with multiple colon polyps is typical for Gardner's syndrome, we have discussed in detail the possiblity of an unknown variant of it. On the other hand, most features of Gardner's syndrome (cutaneous and subcutaneous epidermoid cysts, desmoid tumours, generalized osteomas, a marked multitude of colon polyps, early manifestation of skin and bone changes) were absent in both cases whereas, to our knowledge, in Gardner's syndrome perifollicular fibromas have never been seen. Since perifollicular fibromas are organoid tumours of the mesenchymal hair sheath being clearly defined both clinically and histologically, they must not be confused with the equally well characterized cutaneous tumours of Gardner's syndrome. We are prone to assume that the (irregular?) ASSOCAITION OF MULTIPLE PERIFOLLICULAR FIBROMAS AND colon POLYPS Represents a distinct nosological entity neither identical with Gardner's syndrome nor with any other known dermo-intestinal syndrome. Thus, perifollicular fibromatosis should alert the dermatologist to consider periodic thorough examination for intestinal polyps the more as they may change into malignant growth.
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14/91. Superficial collagenous fibroma: immunohistochemical, ultrastructural, and flow cytometric study of three cases, including one pemphigus vulgaris patient with a dermal mass.

    Collagenous fibroma (desmoplastic fibroblastoma) is an extremely rare benign soft tissue tumor of fibroblastic origin. The majority of reported cases have been located in the deep subcutis, fascia, aponeurosis, or skeletal muscle of the extremities, limb girdles, or head and neck regions. There has been no mention of underlying diseases in patients who developed this tumor. We here report an additional three cases of superficial collagenous fibroma, one of which was a dermal lesion occurring in the abdomen of a 26-year-old male patient with a 5-year history of pemphigus vulgaris prior to development of the tumor. To the best of our knowledge, an association between collagenous fibroma and pemphigus vulgaris has not previously been reported. The remaining two tumors were located in the superficial subcutaneous tissue of the infrascapular area and right foot, respectively. There was no tumor recurrence or metastasis during follow up of 18, 25, and 47 months, respectively. All three tumors were well-circumscribed and unencapsulated without infiltrating borders. Histologically, the common denominator of all three cases was paucicellular proliferation of spindle or stellate fibroblasts enmeshed within an extensively collagenous background. Immunohistochemically, there was diffuse strong staining for vimentin and intense focal reaction for smooth muscle actin in two tumors tested. Electron microscopy revealed features consistent with a fibroblastic or myofibroblastic lineage. flow cytometry in two cases demonstrated a diploid dna content with low S-phase fractions, which correlated with minimal MIB-1 nuclear labeling (less than 1%) and benign behavior of this entity.
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15/91. Rapid recurrence of calcifying fibrous pseudotumor (a case report).

    Calcifying fibrous pseudotumor is a benign tumor-like lesion characterized by the presence of a whorl bundle of collagen fiber with deposit calcification and infiltration of inflammatory cells. This lesion is reportedly rare and involves subcutaneous or deep soft tissue in the extremities, trunk, neck and so on. This tumor has low recurrence even if only simple excision or enucleation is performed. We report a case of calcifying fibrous pseudotumor arising in the nuchal lesion of an 8-year-old boy who had a local recurrence 18 months after a simple excision had been performed. The patient had no evidence of recurrence or metastasis 10 months after the second operation.
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16/91. Pseudosarcomas of the pharynx and larynx.

    Pseudosarcoma of the pharynx and larynx has remained an enigma both to the pathologist and clinician since its original description by Lane in 1957. These lesions are usually polypoid growths that microscopically present very bizarre appearances beneath a squamous cell carcinoma. Eight cases of pseudosarcoma of the larynxand pharynx were observed. These occurred almost twice as often in males as females, with an average age of 56 years. Most were extralaryngeal lesions, and in all cases surgical excision was the primary therapeutic modality. A thorough review of the literature summarizes the clinical and pathologic characteristics of these lesions. There is strong clinical evidence against the traditional belief of the benign course of these lesions, and we recommend that they be treated aggresively like any other carcinoma of the head and neck.
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17/91. Extra-abdominal fibromatosis: a case report.

    head and neck fibromatosis is a rare disease with a variable clinical picture. We report the case of a 40-year-old woman who had a 1-week history of a painless mass overlying her right neck. histology confirmed a diagnosis of extra-abdominal fibromatosis. Two unusual features of this case were the site of the lesion and its rapid growth. In this article, we discuss the clinical picture of head and neck fibromatosis, its etiology and pathology, and current opinion regarding its treatment.
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18/91. Ossifying fibromyxoid tumour of soft parts, with focus on unusual clinicopathological features.

    AIMS: Ossifying fibromyxoid tumour is a rare lesion that generally occurs in the soft tissues of proximal limbs/girdle, or head and neck. Histologically, it usually consists of bland, mitotically inactive, S100 cells in a fibromyxoid matrix with a characteristic peripheral ossification. However, we present two cases that deviated from this pattern. MATERIAL AND methods: One tumour, which was removed from the axilla of a 55-year-old female, was unusual in its large size (120 mm in diameter) and in its restriction of bone formation to its central zones. The other tumour, which occurred in a 62-year-old female, was remarkable in its acral location on the hand and high mitotic activity (four mitoses per high-power field). These features caused concern and follow-up of the patients was recommended. So far there have been no recurrences (25 and 18 months after surgery, respectively). An additional previously undescribed feature was the presence of fibronexus-like structures by electron microscopy in the second case. However, a myofibroblastic phenotype was not supported by the immunohistochemical findings. CONCLUSION: Though a distinct entity, ossifying fibromyxoid tumour may exhibit a wider clinicopathological spectrum than generally recognized.
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19/91. Widely invasive solitary fibrous tumor of the sphenoid sinus, cavernous sinus, and pituitary fossa.

    Solitary fibrous tumor is a spindle cell tumor first described in the pleura, but also found in multiple extrathoracic sites including the meninges, orbit, nasal and paranasal sinuses. No cases have been previously reported in the cavernous sinus or pituitary fossa. We present the case of a 54-year-old woman who presented with progressive amaurosis. On imaging studies, a widely infiltrative lesion involving the pituitary fossa, sphenoid sinus, cavernous sinus, carotid artery, medial temporal, ethmoid, and pterygoid bones, and extending into the nasopharynx was discovered; impression was a malignant tumor originating in the pituitary fossa. At surgery, the tumor was only partially resectable because of extensive bony invasion and encasement of the carotid artery, and was found to compress but not invade the pituitary gland. histology showed a spindle cell proliferation with a dense, hyalinized collagenous stroma and dilated vascular spaces, some showing a staghorn-like appearance. Areas of cellular pleomorphism and increased cellularity were present, but few mitoses were identified. immunohistochemistry showed strong positivity with CD34, factor xiiia, CD99, and Bcl-2. There was scattered cyclin d1, mib-1, and p53 positivity. Muscle, epithelial, vascular, and melanocytic markers were negative. These results led to the diagnosis of solitary fibrous tumor. The size, extensive invasion, and bony destruction indicated a tumor with at least low malignant potential. The occurrence of solitary fibrous tumors in the pituitary fossa and sinuses of the head and neck is rare, but must be considered in the differential diagnosis of spindle cell lesions in these regions.
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20/91. Malignant solitary fibrous tumor of the tongue.

    Solitary fibrous tumor is a generally benign spindle-cell neoplasm that has been predominantly described in the visceral pleura and other serosal sites and is extremely rare in the head and neck area. We report the first known case of malignant solitary fibrous tumor of the tongue in a 57-year-old female patient who experienced rapid growth of a longstanding right anterior tongue mass, with associated dysphagia and dysarthria. magnetic resonance imaging was suggestive of a low-flow vascular malformation not requiring preoperative embolization. The patient underwent partial peroral glossectomy for the excision of the tumor. Final pathology, however, was consistent with solitary fibrous tumor, characterized as malignant by histopathologic criteria, and the patient was returned for re-excision of the close surgical margins. The patient has experienced symptomatic improvement, and she remains free of recurrence 12 months later. All reports of solitary fibrous tumors of the oral cavity and 3 reports of the tongue tumors described in the literature to date represent benign lesions. To our knowledge, this is the first report of a malignant solitary fibrous tumor of the tongue. Presentation, differential diagnosis, criteria for malignancy, treatment, and possible prognostic implications of this rare entity are discussed.
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