Prevention and treatment of injection-site sarcomas.
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Animals↗
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Results for “Dermatofibrosarcoma”
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Dorsal hand coverage with free serratus fascia flap.
In reconstructing a defect on the dorsum of the hand, with the extensor tendons exposed or even missing, functional, as well as cosmetic, goals are of major importance. The authors present three cases of extensor tendon reconstruction, combined with soft-tissue reconstruction, with the free serratus fascia flap, the connective tissue over the serratus muscle, for dorsal hand coverage. The flap consists of thin and well-vascularized pliable tissue, with gliding properties excellent for covering exposed tendons. It is based on the branches of the thoracodorsal artery, which are raised in the flap, leaving the long thoracic nerve intact on the serratus muscle. Coverage of the flap with split-thickness skin graft is done immediately. The free serratus fascia flap is an ideal flap for dorsal hand coverage when the extensor tendons are exposed, especially because of low donor-site morbidity.
Adult↗
Giant-cell fibroblastoma and dermato fibro sarcoma protuberans: the same tumoral spectrum? Report of two cases of association in children.
We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.
Cell Transformation, Neoplastic↗
Medical pearl: new views through the microscope.
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Biomarkers↗
Bednar tumor: report of a case with immunohistochemical and ultrastructural study.
A slowly growing tumor in the right shoulder of a 38-year-old white male, which felt like a superficial cystic mass, was studied. The spindle cells, which represented the main component of the tumor, were arranged in a typical storiform pattern and were positive for CD34 and focally for CD117. The pigmented cells were mostly found at the center of the storiform whorls and were negative for S-100 protein and HMB-45. Ultrastructurally, the tumor consisted predominantly of nondescript mesenchymal spindle cells that resembled fibroblasts. The tumor cells blended into a loosely arranged stromal tissue background. The general appearance of pigmented cells was very similar to the nonpigmented spindle cells. The pigment appeared to be a mature form of melanin granules. The lack of premelanosomes, cell injections, basal lamina, and pinocytotic vesicles was inconsistent with a neural origin/neural differentiation hypothesis for this tumor.
Adult↗
Giant dermatofibroma with monster cells.
We report a case of a 64-year-old woman with a giant dermatofibroma on her back with the unusual histologic feature of monster cells. The firm, exophytic, 3-cm nodule had purple and yellow components with surface telangiectasia. Histologic examination demonstrated characteristic findings of a dermatofibroma, including rete ridge flattening and bridging; a stroma containing scattered, large, round, eosinophilic collagen bundles; and a polymorphous dermal infiltrate of spindle and xanthomatous cells with scattered siderophages. Some xanthomatous cells demonstrated features consistent with monster cells, including huge bizarre nuclei and one or more nucleoli. Immunohistochemical staining for factor XIIIa was positive. A diagnosis of giant dermatofibroma with monster cells (DFMC) was made. Giant dermatofibromas are rare, with monster cells being an uncommon finding in dermatofibroma. To our knowledge, this is the first report of DFMC.
Carcinoma, Squamous Cell↗
S100-positive spindle cells in scars: a diagnostic pitfall in the re-excision of desmoplastic melanoma.
Distinguishing desmoplastic melanoma (DM) from scar tissue on routine microscopy can be difficult, especially in re-excision specimens, and S100 immunohistochemistry has been recommended as a useful adjunct. The purpose of this study is to evaluate the extent and nature of S100 positivity in scars. In this study, formalin-fixed paraffin archival tissues were evaluated with immunohistochemistry. Ten re-excision specimens of previously biopsied nonnevomelanocytic lesions were immunostained with the S100 and CD57 (Leu 7) antibodies. In 9 of the 10 cases, the scars contained S100-positive spindle cells, but there were no cases with CD57+ cells. Ten re-excised atypical nevi and 10 re-excised melanomas were also immunostained for the S100 protein, and all 20 cases contained S100-positive spindle cells within the scars. There was a trend toward quantitatively more S100-positive spindle cells in these nevomelanocytic re-excisions. To evaluate the nature of the spindle cells, scars from two of the nonnevomelanocytic re-excisions were further analyzed utilizing immunostains for glial fibrillary acidic protein, HMB-45, Melan-A, CD1a, factor XIIIa, and neuron specific enolase. In both scars, neuron specific enolase diffusely stained the fibroblast population, but the remaining immunostains were negative in the scar. The presence of S100-positive spindle cells in scars represents a potential diagnostic pitfall, particularly in the evaluation of re-excision specimens of DM.
Cell Count↗
Myxofibrosarcoma presenting in the skin: clinicopathological features and differential diagnosis with cutaneous myxoid neoplasms.
Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) is one of the most common fibroblastic sarcomas in the older patient, where it can sometimes present with anatomically deceptive boundaries. Myxofibrosarcoma is now fully characterized as a distinctive and definable pathologic entity. Clinically there is a tendency for predominantly subcutaneous, multinodular, diffusely infiltrative growth, which may extend to the overlying dermis and present as a cutaneous lesion. Histologically myxofibrosarcoma comprises a spectrum ranging from hypocellular low-grade myxoid to high-grade pleomorphic sarcoma. We report herein 6 cases of myxofibrosarcoma each with dermatological presentation as a cutaneous nodule. The dermal component in each of the lesions was low- to intermediate-grade and predominantly myxoid resulting in confusion with benign myxoid neoplasms in small biopsy specimens. The purpose of this series is to focus the attention of workers in dermatology on a subject rarely discussed in dermatopathology literature: the cutaneous presentation of myxofibrosarcoma and the potential for clinical and histologic misinterpretation, as benign dermal lesions.
Actins↗
CD34 expression in neural and fibrohistiocytic lesions.
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Antigens, CD↗
Upper limb reconstruction with reverse flaps: a review of 52 patients with emphasis on flap selection.
Reverse flaps lend themselves to transposition from proximal to distal locations in the extremities. This series comprised 18 radial forearm flaps, 17 digital artery flaps, 13 posterior interosseous flaps, 3 lateral arm flaps, 2 dorsal digital flaps, and 1 dorsal metacarpal flap, all of which were utilized in a reverse pattern. The radial forearm flap was mainly chosen for defects involving part of the palm and the palmar aspect of the first web space. The posterior interosseous flap was more commonly utilized for resurfacing the dorsum, dorsal aspect of the first web space, and especially the hypothenar aspect of the hand. The lateral arm flap was used to reconstruct antecubital fossa and proximal forearm defects. All posterior interosseous and lateral arm donor areas were closed primarily. Sensate digital artery flaps yielded 5 mm on average static two-point discrimination in 6 to 18 months of follow-up. Functional and cosmetic results concerning the recipient and donor areas were found to be satisfactory. It was concluded that reverse flaps are versatile tools in the coverage of all kind of defects in the upper limb and should be thought of in the first place.
Adolescent↗
Composite cervicofacial flap for reconstruction of complex cheek defects.
The authors present the reconstructive technique for complex cheek defects using the composite cervicofacial flap and study the possibilities, advantages, disadvantages, and results that can be expected. The design follows the classic outline of Mustardé's flap. The skin is undermined for 2 cm anterior to the ear, then after incision of the superficial musculoaponeurotic system (SMAS), undermining is continued below the plane of the SMAS, level with the facial nerve branches. It is continued forward to the facial vessels, which give rise to branches that ensure the blood supply of this composite flap and contribute to its high reliability. In the cervical region, undermining is done beneath the platysma, which is transected transversely in the lower cervical region to allow good upward mobility and satisfactory transposition of the flap. The flap is adapted to the defect and the medial suture line is placed as near as possible to the medial limit of the cheek aesthetic unit. The authors carried out a retrospective study of 7 patients with complex facial reconstruction after excision of malignant lesions. The defects measured from 4x4 cm to 9x7 cm. In 4 patients excision included the periosteum, and in 1 patient excision involved the entire thickness and removed the entire anterior half of the cheek. In 4 patients reconstruction involved the cheek and eyelid. In spite of the advanced age of the patients (88, 69, 91, 67, 70, 82, and 59 years), there was no distal edge necrosis. The only complication was a single case of facial paresis, which resolved spontaneously. The results were considered very good in all 7 patients. The authors conclude that the composite flap increases the possibilities of the cervicofacial flap. It is more mobile, more reliable, thicker, and more adaptable. It can be used in complex cheek defects that involve the periosteum, or even in full-thickness defects. The quality of the results obtained using this flap represents a considerable advance in facial reconstruction.
Aged↗
Free flaps in the treatment of locally advanced malignancy of the scalp and forehead.
Locally advanced cutaneous malignancy of the scalp and forehead is a disease that requires an aggressive approach to resection and reconstruction. Free flap reconstruction in these sites has been advocated because of the advantages of importing large amounts of well-vascularized tissue into a recipient site, which has often been compromised by previous surgery or radiotherapy. A consecutive series of 32 free flap reconstructions in 29 patients with cutaneous malignancy of the scalp and forehead was reviewed. The flap failure rate was 6% (two flaps) and the major complication rate was 10%. Of the surviving flaps, 97% (N = 29) were successful in reconstructing a challenging group of defects. Three patients developed local recurrence of the primary malignancy (mean follow-up, 21 months). The use of a broad repertoire of free tissue transfers in reconstruction of the scalp and forehead defects has allowed effective treatment of locally advanced malignancy of this region. Critical analysis of the results, however, indicates that microsurgical reconstruction is not without morbidity and that there are refinements in the diagnostic and operative steps of management that can maximize the functional and aesthetic results.
Adult↗
Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases.
Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma. In this study, we report 6 cases of a primitive myxoid mesenchymal tumor of infancy (PMMTI), which previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma or infantile fibromatosis. PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass. All patients were otherwise healthy. The tumors occurred on the trunk, extremities, and head and neck. Grossly, the tumors were nonencapsulated and had a multinodular appearance with focal infiltrative growth, a white fleshy cut surface, and a tumor diameter ranging from 2 to 15 cm. Histologically, a diffuse growth of primitive spindle, polygonal, and round cells occurred in a myxoid background. The tumor cells were arranged in a vaguely nodular pattern with peripheral collagenized stroma, higher cellularity at the periphery, and a delicate vascular network in the background. Immunohistochemically, the tumors displayed diffuse reactivity for vimentin and no reactivity for smooth muscle actin, muscle specific actin, desmin, S-100 protein, or myogenin. Electron microscopy documented a poorly differentiated fibroblastic proliferation. Four cases tested negative for the ETV6-NTRK3 gene fusion by RT-PCR. One tumor had a complex karyotypic abnormality with rearrangements involving chromosomes Y, 9, and 3. Three patients had recurrences or metastasis treated with a combination of surgery and chemotherapy. One patient is alive with persistent locally aggressive disease, 2 are alive with no evidence of recurrence, 1 had a recurrence treated surgically without further follow-up information, 1 patient died with persistent tumor and sepsis 6 weeks after diagnosis, and 1 patient was lost to follow-up. The morphologic appearance combined with the ultrastructural features and absence of the typical gene rearrangement of congenital-infantile fibrosarcoma are unique, and we propose that PMMTI represents a new category of pediatric fibroblastic-myofibroblastic tumor.
Dermatofibrosarcoma↗
Two options for perforator flaps in the flank donor site: latissimus dorsi and thoracodorsal perforator flaps.
Two types of perforators, septocutaneous and musculocutaneous, are found in the same donor site of the flank area, and two perforator flaps based on each perforator are clinically available. Therefore, it is necessary to distinguish them from one another using different nomenclatures. Accordingly, the perforator flap based on a musculocutaneous perforator is named according to the name of the muscle perforated, the latissimus dorsi perforator flap, and the perforator flap based on a septocutaneous perforator, located between the serratus anterior and latissimus dorsi muscles, is named according to the name of the proximal vessel, the thoracodorsal perforator flap. In this series of 42 latissimus dorsi perforator flaps, flap size ranged from 5 x 3 cm to 20 x 15 cm, and two complications were observed: a marginal necrosis in an extremely large flap (26 x 12 cm) and a failure caused by infection. The thoracodorsal perforator flap was used in 14 cases, including two cases of chimeric composition. Flap size ranged from 4.5 x 3.5 to 18 x 15 cm, with no complications. In the two patterns of perforator flap that the author used, initial temporary flap congestion was observed in five latissimus dorsi perforator flap cases and two thoracodorsal perforator flap cases, when the flap was designed as a large flap or a less reliable perforator was selected. However, the congestion was not serious enough to cause flap necrosis. Several techniques, such as T anastomosis or inclusion of an additional perforator or a small portion of muscle, are recommended to prevent the initial flap congestion, especially when an unreliable perforator is inevitably used or when a flap larger than 20 cm long is required. A small portion of the muscle was included in six cases, when an unduly large or improperly long flap was planned. All of the flaps were successful and ranged from 22 x 7 to 15 x 28 cm, except for one case of distal flap necrosis in an extraordinarily large flap measuring 34 x 10 cm. Diverse selection of the perforator flap is one of the great advantages of the flank donor site, providing it with wider availability and more versatile composition for reconstruction or resurfacing.
Adolescent↗
Detection of COL1A1-PDGFB fusion transcripts and platelet-derived growth factor alpha and beta receptors in giant cell fibroblastoma of the postsacrococcygeal region.
We describe a 2-year-old girl with recurrent giant cell fibroblastoma (GCF) of the postsacrococcygeal region. Both the initial and recurrent tumours contained solid and angiectoid areas. The former was composed of loosely arranged wavy spindle cells and giant cells with a well-vascularized myxoid to collagenous stroma. The angiectoid spaces were often lined by multinucleated giant cells. Immunohistochemically, the tumour cells and small vessels in the tumour tissue were positive for platelet-derived growth factor (PDGF) alpha and beta receptors. Molecular analysis revealed fusion of collagen type Ialpha1 exon 26 with PDGF-B chain exon 2 that induced unscheduled production of PDGF-BB. These findings suggest that PDGF and its receptors significantly contribute to the development of GCF in both an autocrine and a paracrine manner.
Base Sequence↗
Linear dermatomyofibroma.
Dermatomyofibroma is a rare, plaque-like, cutaneous myofibroblastic proliferation that usually presents as a 1-2 cm, firm, dermal plaque, often with brown-red discoloration. We report an example of dermatomyofibroma presenting as a poorly defined, linear, indurated area, 8 cm in length, on the posterior aspect of the neck of a 20-year-old female. This lesion is the largest dermatomyofibroma reported to date and the first to demonstrate a linear distribution.
Adult↗