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PubMed1986

Pseudotumor of the supraclavicular fossa.

Minor musculoskeletal variants of the upper thoracic spine or of the ribs can cause effacement of the supraclavicular fossa, simulating a mass ("pseudotumor"). These variants may occur singly or in combination, and include unilateral prominence or asymmetry of the first rib, unilateral prominence or asymmetry of a cervical rib, and mild upper thoracic scoliosis causing prominence of the first rib on one side. The detection of variants by physical examination or more easily by review of the chest x-ray film may in selected instances obviate the need for costly diagnostic procedures and unnecessary biopsies. We call attention to this condition and describe our experience with four patients.

Aged↗
PubMed1992

Large soft tissue tumor of the chest occurring after trauma.

The unusual soft tissue tumor we have described may be the largest yet reported. The tumor was considered benign, and its histologic features were most consistent with fibromatosis arising in a region that had been previously traumatized. Although the patient is doing well, it is premature (2 years later) to conclude that the tumor will not recur.

Adolescent↗
PubMed1990

Primary tumors of the axial skeleton. Experience of the Leeds Regional Bone Tumor Registry.

Primary tumors of the axial skeleton are rare and a survey of the Leeds Regional Bone Tumor Registry found them to constitute only 55 of the 1950 cases (2.8%). Chordoma was the most frequent tumor in the cervical and sacral regions as well as the most common diagnosis overall and osteosarcoma ranked second. Pain was the most frequent presenting symptom but over half the patients developed some neurological abnormality. In spite of treatment survival was poor in patients with malignant lesions or neurological involvement. The establishment of Bone Tumor Registries is the only way that sufficient data on large numbers of these rare tumours can be amassed to provide a valuable and otherwise unavailable source of information for research, education and service.

Adolescent↗
PubMedDate not supplied

Treatment of childhood lymphangiomas with interferon-alpha.

PURPOSE: Nonsurgical treatment of lymphangiomas has shown limited efficacy and often carries unacceptable toxicities, demonstrating the need for a more effective, less toxic therapy. PATIENTS AND METHODS: We describe two patients with lymphangiomatosis treated for 12 to 40 months with recombinant interferon-alpha. RESULTS: Both patients demonstrated stabilization or marked improvement of disease, based on clinical and radiologic findings, with minimal toxicity. CONCLUSIONS: The favorable responses to interferon-alpha therapy in these two cases suggest that this is an effective and well-tolerated treatment for lymphangiomas in children.

Adolescent↗
PubMedDate not supplied

Cerebral metastases of alveolar rhabdomyosarcoma in an infant with multiple skin nodules.

PURPOSE: This report describes extremely rare cases of infantile rhabdomyosarcoma with multiple skin nodules. They are of interest not only for their anatomic sites, but also for subsequent cerebral metastases with sudden cranial hypertension. PATIENTS: Two infants had multiple skin nodules and excisional biopsy revealed alveolar type rhabdomyosarcomas. The patients were treated with tumor resection and combined chemotherapy without any clinical progression for 9 and 16 months, respectively. RESULTS: Evidence of cerebral metastases developed with sudden vomiting and convulsion as the first manifestation. In one patient, urgent radiographic examinations failed to reveal lesions except for dilated cerebral ventricles. Seven weeks after the onset of the neurologic symptoms, only Gd-DPTA-enhanced magnetic resonance imaging (MRI) revealed multiple punctate metastatic lesions hyperintense to the surrounding cerebral tissue. Despite appropriate chemotherapy, both patients had disease progression and died of central nervous system metastases. CONCLUSIONS: The authors emphasize the need to recognize the multiple cutaneous presentation of infantile rhabdomyosarcoma and the association of cerebral metastases as a potential and fatal complication. The diagnosis is facilitated by Gd-DPTA-enhanced MRI, particularly when cerebral computed tomography scans fail to disclose metastatic lesions.

Biopsy↗
PubMed2005

Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(X;18)-positive cases from the French Sarcoma Group and the Mesopath Group.

Synovial sarcoma (SS), an aggressive neoplasm accounting for up to 14% of soft tissue sarcomas, was recently recognized as a primary tumor in the lung and pleura. SS is characterized by the chromosomal translocation t(X;18)(SYT-SSX) found in more than 95% of the tumors. We report a cooperative study from the French Sarcoma Group and the Mesopath Group on 40 t(X;18)(SYT-SSX)-positive primary intrathoracic SS. There were 22 males and 18 females, whose age ranged from 16 to 79 years (median, 47 years). Neoplasms were mostly circumscribed and of large size (median, 7.5 cm; range, 2-16 cm). Thirty-nine tumors were monophasic SS, including 24 (60%) monophasic fibrous and 15 (37.5%) poorly differentiated cases, and one lesion was a biphasic SS. A larger proportion of poorly differentiated tumors were observed among intrathoracic SS as compared with soft tissue SS. Immunohistochemically, 90% of the cases reacted with at least one epithelial marker. CD34 was focally expressed in 3 cases. SYT-SSX1 fusion transcripts were detected in 22 cases (56.4%) and SYT-SSX2 fusion transcripts in 17 cases. Median and 5-year disease-specific survival in 33 patients was 50 months and 31.6%. Median and 5-year disease-free survival was 24 months and 20.9%. Patient sex, age, tumor size, histologic subtype, grade, and SYS-SSX fusion type had no significant impact on outcome. In conclusion, intrathoracic SS are rare but aggressive tumors with poor prognosis. In this unusual location, the detection of SYT-SSX fusion transcripts is a valuable diagnostic adjunct.

Adolescent↗
PubMed2005

Magnetic resonance imaging of the body trunk using a single-slab, 3-dimensional, T2-weighted turbo-spin-echo sequence with high sampling efficiency (SPACE) for high spatial resolution imaging: initial clinical experiences.

PURPOSE: The authors conducted a clinical evaluation of single-slab, 3-dimensional, T2-weighted turbo-spin-echo (TSE) with high sampling efficiency (SPACE) for high isotropic body imaging with large field-of-view (FoV). MATERIALS AND METHODS: Fifty patients were examined in clinical routine with SPACE (regions of interest: pelvis n=30, lower spine n=12, upper spine n=6, extremities n=4) at 1.5 T. For achieving a high sampling efficiency, parallel imaging, high turbofactor, and magnetization restore pulses were used. In contrast to a conventional TSE imaging technique with constant flip angle refocusing, the refocusing pulse train of the SPACE sequence consists of variable flip angle radiofrequency pulses along the echo train. RESULTS: Signal-to-noise ratio and contrast-to-noise ratio of SPACE images were of sufficient diagnostic value. The possibility of image reconstruction in multiple planes was of clinical relevance in all cases and simplified data analysis. CONCLUSION: The achievement of 3-dimensional, T2-weighted TSE magnetic resonance imaging with isotropic and high spatial resolution and interactive 3-dimensional visualization essentially improve the diagnostic potential of magnetic resonance imaging.

Abdominal Neoplasms↗
PubMed2004

Chest wall reconstruction using iliac bone allografts and muscle flaps.

Technically we can divide full-thickness thoracic reconstruction into 2 parts: providing a rigid support and ensuring well-vascularized coverage. Since 1986, the authors' center has had ample experience with bone banks and the use of cryopreserved bone grafts, which led them to consider the possibility of using these grafts for full-thickness chest wall reconstruction. They describe 3 patients in whom resection of the tumor and reconstruction of the thorax were carried out using iliac bone allografts covered with muscle flaps (1 pectoralis major and 2 rectus abdominis). None of the patients experienced breathing difficulties, pain, or instability after 14 months, 18 months, and 11 years of follow-up. The result of the reconstruction was excellent in all 3 patients in terms of function and aesthetics. The advantage of allografts compared with synthetic materials is their potential integration; they can become part of the host patient's living tissue.

Adult↗
PubMed2006

Never say no to a damaged muscle: the applications of previously damaged pectoralis major and latissimus dorsi muscles for chest wall reconstruction.

The nondamaged latissimus dorsi and pectoralis major muscles are widely reported in the literature as being the most frequently used for chest wall reconstruction. In this study, we report a series of 9 patients with full-thickness chest wall defects who underwent reconstruction with previously damaged latissimus dorsi or pectoralis major muscles. Nine patients underwent chest wall reconstruction over a period of 3 years using previously damaged latissimus dorsi or pectoralis major muscles. The thoracic defect was posttraumatic in 3 patients and post-tumor resection in 6 patients. Three patients had previous radiation therapy. In 8 cases, the full-thickness chest wall defects were completely closed. The only failure encountered was in one patient who had a long lasting fistula. The proximal based island flap that was inserted into the tract showed good vascular supply and viability; however, it did not solve the clinical problem. Keeping in mind the basic rule of medicine ("Primum Non Nocere") and a thorough knowledge of the surgical anatomy of these muscles are essential for the successful application of these previously damaged muscles for chest wall reconstruction.

Humans↗
PubMed2005

Giant congenital melanocytic nevi of the trunk and an algorithm for treatment.

Giant congenital melanocytic nevi (CMN) are rare, congenital, disfiguring lesions with a risk of degeneration to malignant melanoma. Giant CMN are associated with an increased risk of malignant degeneration. In a minority of cases, patients with giant CMN may have associated neurocutaneous melanosis with leptomeningeal involvement. Giant CMN of the trunk pose difficult diagnostic and reconstructive problems requiring complex multistage treatment. For high-risk cases, diagnostic evaluation in the form of neuro-imaging is an essential component of the planning phase. Although nonsurgical options for the treatment of giant CMN have been advocated, these modalities may decrease the burden of nevus cells but do not result in complete removal of these cells. The ability to monitor nevus cells that remain after nonsurgical management of giant CMN remains questionable. These nonsurgical options include dermabrasion, laser ablation, and chemical peel. In contrast, direct excision of the nevus is the mainstay of treatment of nonsurgical management of giant CMN. There are numerous surgical options to resurface the resultant cutaneous defect after excision of the nevus. The simplest of these options consists of serial excision and direct closure of the defect in stages. However, if the defect cannot be closed by direct cutaneous advancement, other options for wound resurfacing include split- or full-thickness skin graft, tissue expansion, and free tissue transfer. Tissue expansion should be viewed as a category of treatment options because expanders can be used to create an expanded full-thickness skin graft, local expanded flaps adjacent to the lesion, or expansion of a free tissue donor site. Given the diversity of reconstructive options that use tissue expansion, these techniques have evolved as the primary treatment method for giant CMN of the trunk. The authors outline an approach to the evaluation of giant CMN of the trunk, review the risks of melanoma and of neurocutaneous melanosis, describe their preferred treatment regimen, and offer a treatment algorithm for giant CMN of the trunk.

Algorithms↗
PubMed2006

Two extreme conditions of xeroderma pigmentosum.

Two atypical cases of xeroderma pigmentosum are reported. One of these cases with lung metastasis of squamous cell carcinoma in the first decade of life and the other with late onset disease (XP) in fifth decade of life. Lung metastasis of squamous cell carcinoma in first decade of life in patients with xeroderma pigmentosum has never been reported in the literature to our knowledge. Late onset of the disease in fifth decade is also a rare condition. Clinicians should be more alert for the internal organ metastases to improve the survival of the patients and should never forget that xeroderma pigmentosum has got a wide range of clinical presentation.

Carcinoma, Squamous Cell↗
PubMed2005

Pedunculated localized fibrous tumor of the pleura presenting as a moving chest mass.

Localized fibrous tumor of the pleura is a rare primary pleural neoplasm. The tumors are usually discovered incidentally on routine chest radiography. A change in the lesion's position with respiration and/or posture of the patient indicates a pedunculated pleural lesion. Computed tomography and magnetic resonance imaging reveal the relation of the lesions to adjacent structures. Magnetic resonance imaging is also useful for the characterization of the lesions on the basis of signal features. These tumors usually consist of mature fibrous tissue.

Female↗
PubMed1997

Truncal tumor site is associated with high risk of multiple basal cell carcinoma and is influenced by glutathione S-transferase, GSTT1, and cytochrome P450, CYP1A1 genotypes, and their interaction.

Basal cell carcinoma (BCC) places increasing burdens on clinicians; incidence is rising and patients may develop multiple primary tumors. Although UV exposure is critical, many patients develop tumors at less-exposed sites, such as the trunk, suggesting a genetic predisposition. We previously showed that polymorphism in loci encoding the detoxifying enzymes, glutathione S-transferase (GSTM1, GSTM3, GSTT1) and cytochrome P450 (CYP2D6, CYP1A1) influences susceptibility to BCC. We now describe a case-control approach in 345 patients with BCC that examines the role of these polymorphisms and patient characteristics (age, gender, skin type, hair color, eye color, smoking, occupation) in determining susceptibility to truncal tumors. GST and CYP genotypes were identified using polymerase chain reaction-based methods. Patients with one or more truncal tumors were significantly younger (p = 0.0170) than those with no truncal tumors. Male gender also appeared more common in the truncal tumor group, although this did not achieve significance (p = 0.0925). Patients whose first tumor was truncal had significantly more tumors (p = 0.0297). GSTT1 null (p = 0.0245, odds ratio 2.24) and CYP1A1 Ile/Ile (p = 0.0386, odds ratio 2.86) were associated with truncal site after correction for age and gender. The combination, GSTT1 null and CYP1A1 Ile/Ile, was particularly significant (p = 0.0059, odds ratio = 2.95). These effects were present after correction for tumor numbers. These data show first, patients with truncal tumors constitute a high-risk group for BCC, second, a significant genetic influence on BCC site, and third, a significant interaction between GSTT1 and CYP1A1 genotypes.

Aged↗
PubMed1995

Successful treatment of dark-colored epidermal nevus with ruby laser.

The pulsed ruby laser has a selective thermolytic effect. Recently, it has been available for the treatment of superficial pigmented disorders. We studied 5 cases of epidermal nevus treated with the pulsed ruby laser. In comparison with the usual methods including electrocautery, cryotherapy and skin abrasion, ruby laser therapy is an excellent tool due to technological ease and rapid improvement. Depigmentation after treatment in 2 cases was the only side effect of this therapy. Bose cases had a dark pigmentation of the skin. Despite of the risk of discoloration, the ruby laser is one of the most effective tools for therapy of pigmented epidermal nevus.

Adolescent↗
PubMed1987

Soft tissue gliomatosis--heterotopic glial tissue in the subcutis: a case report.

A subcutaneous nodule composed of heterotopic glial tissue is reported. The lesion was present in the subcutaneous tissue of the chest wall in a 2-year-old female. Histologically it was composed of loosely textured fibrillary neuroglial tissue in a densely hyalinized collagenous stroma. Immunohistochemical staining for glial fibrillary acidic protein was positive. The possible origin of this soft tissue glial heterotopia is discussed and the subject of heterotopic glial tissue is reviewed.

Axilla↗
PubMed1993

Divergent differentiation in small round-cell tumours of the soft tissues with neural features--an analysis of 10 cases.

A series of 10 small round-cell tumours, having in common evidence of neural differentiation, were investigated by immunohistochemistry and electronmicroscopy. In seven, evidence of divergent muscle and/or epithelial differentiation was found. This phenomenon thus appears more common than previously appreciated and suggests that there may be a continuous and overlapping phenotypic spectrum from Ewing's tumour of soft tissue to intra-abdominal desmoplastic small cell tumour.

Adolescent↗