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PubMed1984

Osseous destruction by neurofibroma diagnosed in infancy as "desmoplastic fibroma".

A 2-month-old male infant presented with a lytic lesion of the distal radius which was diagnosed as desmoplastic fibroma. Tumor recurred following en bloc resection of the distal radius and forearm, necessitating an elbow disarticulation. Subsequently, the child developed multiple café-au-lait spots, and histologic sections have been reinterpreted as neurofibromatosis. This case illustrates the potential difficulty in diagnosing neurofibromatosis when few or no classic stigmata of the disease are present.

Adult↗
PubMedDate not supplied

Antalgic scoliosis due to nonosteogenic fibroma of the L1 neural arch: report of a case.

A case of nonosteogenic fibroma of the neural arch associated with scoliosis is reported. The unusual location of the neoplastic lesion and its close relationship with the lateral deviation of the spine are discussed. The necessity for accurate, early etiologic diagnosis is stressed. Early resection of the lesion led to resolution of the spinal curvature and pain.

Adolescent↗
PubMed2007

Scintigraphic characteristics of non-ossifying fibroma in military recruits undergoing bone scintigraphy for suspected stress fractures and lower limb pains.

INTRODUCTION: Non-ossifying fibroma (NOF) is the most common fibrous bone lesion in children and young adults. This benign lesion is not a true neoplasm but is considered a developmental defect. Clinically, the lesion is asymptomatic and has a predilection for the long bones, particularly the femur and the tibia. NOF that ossify can show increased uptake on bone scintigraphy. Although the radiographic and histopathological findings of NOF have been well described, the scintigraphic findings of the abnormality have only been incidentally mentioned in the literature. AIM: To document the scintigraphic features of NOF in a group of military recruits undergoing bone scintigraphy for suspected stress fractures. Features to differentiate co-existent NOF and stress fractures lesions are discussed. MATERIALS AND METHODS: Eighty-three military recruits, 67 male and 16 female, aged 18 to 22 years (mean, 19.4 years), who underwent Tc-methylene diphosphonate bone scans for suspected stress fractures or because of pain of the lower limbs had 91 focal lesions on bone scan which on further evaluation demonstrated characteristic radiographic findings of NOF. We evaluated the anatomical site of the lesions, documented the intensity of uptake on bone scan and compared the findings with the radiographic description of the lesions. Comparison with the characteristic scintigraphic pattern of co-existent stress fracture lesions and with previously reported data was performed. RESULTS: A total of 91 NOF lesions were detected. Overall, 89% of NOF were located about the knee. Anatomic distribution of NOF lesions was as follows: 43 (47.3%, R=25, L=18) were located in the postero-medial aspect of the distal femur, 18 (19.8%, R=12, L=6) in the postero-medial aspect of the proximal tibia, 11 (12%, R=5, L=6) in the postero-lateral aspect of the distal femur, 10 (11%, R=4, L=6) in the postero-lateral aspect of the distal tibia, 4 (4.4%, R=2, L=2) in the postero-lateral aspect of the proximal tibia, 3 (3.3%, L=3) in the antero-central aspect of proximal tibia, 1 (1.1%, L=1) in the antero-lateral aspect of distal femur, 1 (1.1%, L=1) in the medial-central aspect of the proximal tibia. In this series NOF lesions were not found in the fibula. Eighty five of 91 (93.4%) of all NOF were located at the metaphysis of the long bones, 2/91 (2.2%) were located at the meta-diaphyseal region of the long bones and only 4/91 (4.4%) of the lesions were located at the diaphysis. All the NOF showed variable degrees of focal increased tracer uptake on bone scan. The bone scan appearance of the focal lesions was: faint uptake in 29 (31.9%), mild uptake in 27 (29.7%), moderate uptake in 28 (30.7%) and intensely increased uptake in seven (7.7%). The radiographic description of the NOF was: lucent NOF three (3.3%), mixed sclerotic and lucent 68 (74.7%) and sclerotic in 20 (22%). Most of the NOF which demonstrated moderate or intensely increased tracer uptake had mixed lucent and sclerotic radiographic appearance (healing). Most of the sclerotic lesions (healed) showed faint uptake. Co-existent stress fractures were predominantly located in the diaphysis of the long bones, characteristically in the postero-medial aspect of the mid-third of the tibia or femur. CONCLUSIONS: Military recruits undergoing bone scan for suspected stress fracture might have incidental findings which require further evaluation. Focal lesions on bone scan located about the knee in the lateral aspect of the distal femur or lateral aspect of the proximal tibia in the metaphyseal region of these bones are not compatible with the characteristic scintigraphic features of stress fracture. Such a finding should raise the suspicion for other bony lesions such as NOF, which is commonly located in this region. During the healing phase of the NOF which commonly occur in the age range of this group, the lesion shows mild-to-moderate increased tracer uptake on bone scan. Plain film radiography is usually diagnostic and patients are followed up conservatively. Some NOF lesions are still indistinguishable from stress fracture or splints on bone scan.

Adolescent↗
PubMed1980

Electron microscopic immunoperoxidase studies on the accumulation of virus antigen in cells infected with Shope fibroma virus.

The indirect immunoperoxidase technique was used to investigate the development of the virus-specific intracellular and cell membrane antigens in cells infected with the Shope fibroma virus. Starting with 6 h p.i., virus antigen formed distinct inclusions within the cytoplasm frequently enclosed by endoplasmic reticulum. The endoplasmic reticulum disappeared almost completely 10 to 12 h p.i., coincidentally with the beginning of virus formation. The virus antigen was distributed throughout the cytoplasm. At the same time virus-induced antigen began to appear at the cell membrane and subsequently increased. No cytochemical staining could be observed on the endoplasmic reticulum, within the nucleus and within immature and mature virus particles. The correlation between antigen synthesis and changes in cell ultrastructure is discussed.

Animals↗
PubMed1992

Nucleotide sequence analysis of a unique near-terminal region of the tumorigenic poxvirus, Shope fibroma virus.

Shope fibroma virus (SFV), a tumorigenic poxvirus, has a DNA genome of approximately 160 kb. Previous DNA sequence analysis of SFV has been mainly limited to the terminal inverted repetitions (about 12 kb at each end of the genome) and immediately adjacent regions. We have sequenced a 4 kb fragment located approximately 20 kb from the right-terminal hairpin. Within this region three complete and two partial open reading frames (ORFs) have been identified. Each of the putative polypeptides has sequence similarity to one or more previously identified poxvirus or cellular proteins, with homology to protein kinases, erythrocyte ankyrin and a vaccinia virus virulence-related protein (ORF N1L). The potential significance of these gene products with regard to the phenotype of SFV is discussed.

Amino Acid Sequence↗
PubMed2006

Pure sclerotic neurofibroma: a neurofibroma mimicking sclerotic fibroma.

BACKGROUND: Neurofibroma (NF) is a benign tumor of the nerve sheath. Several variants of NF have been described. PURPOSE: We report a case of NF with sclerotic changes resembling sclerotic fibroma (SF). METHODS AND RESULTS: The patient was a 61-year-old man who had an asymptomatic cutaneous lesion on the right scapular region. Physical examination revealed a pedunculated, white-to-pinkish nodule that had a firm consistency, spherical morphology, and smooth surface. Microscopically, the nodule showed a well-circumscribed, nonencapsulated dermal tumor composed of scant cells and thick collagen bundles with prominent clefts. The tumor cells were immunoreactive for vimentin and S100 protein. The patient was diagnosed as having pure sclerotic NF. To our knowledge, only five cases of NF with SF-like pattern have been previously published, and in two this pattern was pure. CONCLUSION: It is important to recognize this exceptional type of NF because it may be easily confused with SF, as well as with a wide variety of neoplasms or hamartomatous conditions containing similar sclerotic pattern.

Biomarkers, Tumor↗
PubMed1997

Nuchal fibroma.

Nuchal fibroma was diagnosed in a 54-year-old diabetic woman with a two year history of increased skin thickness of her low-posterior neck and interscapular region, causing discomfort and limitation of neck and arm motion. Physical and laboratory examinations excluded further disorders. The patient was released, free from symptoms, after complete excision of the soft tissue tumor.

Adipose Tissue↗
PubMed1994

Perineurioma (storiform perineurial fibroma): clinico-pathological analysis of four cases.

Four cases of perineurioma (storiform perineurial fibroma) arising in the dermis, subcutis, or deep soft tissue have been studied. Two patients were female and two were male with ages ranging from 19 to 45 years. One lesion each arose on the chest wall, shoulder, neck, and elbow. Follow-up information in three patients revealed no recurrence. Histologically, the neoplasms were circumscribed but non-encapsulated lesions and were composed of spindle cells with elongated bipolar cytoplasmic processes, inconspicuous fusiform nuclei and well-defined palely eosinophilic cytoplasm. These cells were arranged in whorls or lamellar-like structures and often demonstrated a storiform growth pattern. In areas, the tumour cells appeared larger with more rounded nuclei. Immunohistochemically, most of the tumour cells stained positive for epithelial membrane antigen and vimentin, but failed to stain for S-100 protein, neurofilament, desmoplakin, and CD34. Ultrastructurally, two cases showed fusiform tumour cells with long, thin cell processes separated by abundant collagen bundles. Tumour cells were covered by discontinuous external lamina, showed many pinocytic vesicles and occasionally desmosome-like structures. The morphology and EMA immunopositivity of perineurioma are similar to meningioma, especially to cutaneous meningioma type II. We believe that perineurioma and meningioma are closely related, but morphologically distinguishable, neoplasms.

Adult↗
PubMed2006

Peripheral odontogenic fibroma (WHO type) of the newborn: a case report.

AIM: The present paper reports a case of peripheral odontogenic fibroma (POF) (WHO type) in a newborn. The differential diagnosis and treatment were discussed. BACKGROUND: POF is well described in the literature, but this is the first report in a newborn. PATIENT: A 4-month-old female newborn was referred to our department because of an exophytic, sessile, firm, and well-delimited lesion on the right upper alveolar ridge. The covering mucosa was apparently normal. The lesion measuring 10 x 3 mm was present since birth. The clinical diagnosis of congenital granular cell tumour (congenital epulis) or dental lamina cyst of the newborn was made. A conservative excisional biopsy was performed under local anaesthesia, and the specimen was submitted to histopathological examination. RESULTS: The microscopic examination revealed a pattern of POF (WHO type). Normal primary incisors teeth eruption, and no signs of recurrence were noted on 16 months follow-up. CONCLUSION: Despite the rarity of POF in a newborn, this lesion should be included as a possible diagnosis to focal gingival growth.

Alveolar Process↗
PubMed1982

Xanthic variant of non-ossifying fibroma (so-called xanthofibroma) of the mandible. An ultrastructural study.

An unusual case of a 37-year-old female with xanthomatous bone tumor of the right molar area of the mandible was presented. The tumor was asymptomatic and found to be a well-demarcated intraosseous radiolucent lesion on radiographic examination. Histologically the tumor consisted of two cell types, fibroblastic and xanthomatous cells. There was no osteoid, bone or cartilage formation. However, numerous psammomatous calcified bodies were seen in the fibrous area. Ultrastructural study showed fibroblastic cells in different stages of proliferation as the basis of the tumor which transform itself into xanthomatous cells. From the clinicopathologic findings, our case was thought to be a xanthic variant of non-ossifying fibroma (so-called xanthofibroma) of the mandible.

Adult↗
PubMed1985

Fibroma of tendon sheath: a tumor of myofibroblasts. A clinicopathologic study of 18 cases.

A clinicopathologic study of 18 cases of fibroma of tendon sheath included an immunohistochemical survey of 7 cases and an electron-microscopic examination of one. The age of the patients ranged from 1 to 77 years, with a median of 34 years. The most common site of the tumors was the finger (7 cases), followed by the knee (3), the hand (2), and the foot (2). The median greatest diameter of the tumor was 2 cm. The tumors were attached or closely related to the tendon or tendon sheath, and usually well circumscribed, and multinodular or lobulated. Microscopically, spindle or stellate tumor cells with fuchsinophilic cytoplasm were embedded in a dense fibrous stroma with scattered small blood vessels. Most tumor cells have immunoreaction products for actin in the cytoplasm with accentuation along the cell membrane. Ultrastructurally, many of the tumor cells proved to be myofibroblasts.

Actins↗
PubMed1987

Growth potential of peripheral ossifying fibroma.

The peripheral ossifying fibroma (POF), a benign gingival overgrowth, occurs mainly in the anterior portion of the maxilla in young adults. The size of the lesion is usually less than 1.5 cm. A unique case of POF in the posterior mandible of a 70-year-old female is presented. The lesion was 6 cm in the largest diameter. It is apparent that POF can become a sizeable tumor unless it is surgically excised in an early stage.

Aged↗
PubMed1978

Recurrent digital fibroma of childhood.

Four cases of recurrent digital fibroma of childhood are presented, bringing the total of reported cases to 79. From our material and a review of the literature, the most characteristic features of this entity are delineated. There is no predilection for either sex. Seventy-three percent (58 cases) occurred under one year of age with 14 observed at birth; only four patients were over 5-years-old and the oldest was 15. The localization is highly diagnostic, being limited to the second to fifth carpal and pedal digits and no reported involvement of thumbs or great toes. Two cases were observed in the sole and ulnar side of the hand but associated with others in classical locations. In 50% of cases, the lesions are single at the time of diagnosis. The recurrence rate, based upon cases with adequate follow-up, was found to be 75%. To our knowledge no metastasis has ever been reported. The histology is that of well-differentiated fibroblastic tumor with pathognomonic eosinophilic cytoplasmic inclusion bodies. A review of the available electron microscopic data is presented and the histogenesis is discussed.

Child, Preschool↗
PubMed1986

Fibroma of tendon sheath: a clinicopathologic study.

Thirteen cases of fibroma of tendon sheath have been reviewed. This lesion presents predominantly in middle-aged adults, particularly men, and usually arises in the dermis and subcutis of the extremities, most especially the hands. Histologically these tumours are always well circumscribed and may be lobulated. They are largely composed of interlacing bundles of hyalinised, hypocellular fibrous tissue with occasional more cellular areas. A constant feature is the presence of slit-like vascular channels. They are benign lesions with little tendency to recur and, although becoming more generally recognised are not yet familiar to dermatologists.

Adolescent↗
PubMed1990

Trichoblastic fibroma.

We report a trichoblastic fibroma in the groin of a 60-year-old woman. This unusual tumor showed a predominance of epithelial component, with abundant basophilic, well-circumscribed, epithelial lobules and some keratinous cysts. Several areas suggested stromal induction of differentiation, and in the keratinous cysts there were indications of outer hair sheath differentiation. We review the clinical data from published cases of trichogenic hair germ tumors and discuss the differential diagnosis.

Epithelium↗
PubMed1992

Multiple sclerotic fibromas of the skin. A cutaneous marker of Cowden's disease.

Multiple hamartoma syndrome, or Cowden's disease, is a rare genodermatosis with multiple organ system involvement in which malignancy, particularly of breast and thyroid, may develop. Multiple trichilemmomas have been classically regarded as the cutaneous hallmark of this disease. We here emphasize multiple sclerotic fibromas of the skin as another specific cutaneous marker of this entity, which may also be helpful in an early diagnosis.

Adult↗
PubMed1996

Extra-acral calcifying aponeurotic fibroma: a distinctive case with 23-year follow-up.

Calcifying aponeurotic fibroma (CAF) is an unusual but well-characterized soft tissue neoplasm that typically involves the digits of children and frequently recurs locally. This report describes a case from the subcutis of the lumbosacral region. A 26-year-old man initially presented at age 3 and developed three recurrences over a 23-year period each at approximately 8-year intervals (ages 10, 18, and 26). The microscopic findings in all recurrences showed a lobulated, poorly circumscribed proliferation of dense fibrous tissue containing epithelioid-like fibroblasts, multinucleated cells, and islands of metaplastic chondroid differentiation with focal calcification. Despite origin from an unusual anatomic site, this case reported herein demonstrates the classical morphologic features and clinical history of CAF which showed little in the way of morphologic evolution despite 23 years of persistence. The clinical and histologic features helpful in distinguishing CAF from infantile fibromatosis and soft tissue chondroma are discussed.

Adult↗
PubMed1989

Ossifying fibroma of the mandible and maxilla: review of 18 cases.

A clinical, radiographic, histologic and follow up study of 18 cases of ossifying fibroma involving the mandible and maxilla was performed. Differences in behavior and management according to age, location, radiographic appearance and histopathology were not evident. A wide range of microscopic findings within and between cases was evident, while the radiographic finding consisting of sharp delineation was consistent in all cases. The presence or absence of cementum or cementum-like material and various types of formed elements was likewise of little clinical significance. Finally, a potential source of the neoplastic cell from the periodontal apparatus may help explain the range of histologic findings.

Adolescent↗