A 10-year analysis of cutaneous mesenchymal tumors (sarcomas and related entities) in a skin cancer center.

BACKGROUND: Mesenchymal neoplasms (sarcomas) of skin are rare. Patients with sarcomas were analyzed over the last decade. METHODS: Over a 10-year period, we conducted a retrospective analysis of patients diagnosed and treated in an urban academic teaching hospital in Saxony, Germany. Clinical and pathologic files were used. RESULTS: We identified 65 adult patients with 67 primary cutaneous sarcomas. The mean age was 73.1 (± 15.5) years with a male predominance (78.5%). None of the sarcomas was detected by a skin cancer screening program. The diagnosis was atypical fibroxanthoma (n = 41 patients with 43 tumors), cutaneous angiosarcoma (eight), dermatofibrosarcoma protuberans (two), nodular epithelioid cell sarcoma (one), Kaposi sarcoma (three), leiomyosarcoma (five), malignant fibrous histiocytoma (two), fibromyxoid sarcoma (one), and cutaneous angiomyxoma (two). The preferred tumor localization was the head and neck area (44 patients). Follow-up was 0.5-5.5 years (mean 18 ± 12 months). We observed metastatic spread of atypical fibroxanthoma in 12.5%, demonstrating that this type of sarcoma can run an aggressive course. Mohs surgery is still the cornerstone of treatment, although new options in palliative or adjuvant treatment are available. CONCLUSIONS: Mesenchymal neoplasms (sarcomas) are an important group of cutaneous malignancies. Awareness needs to be improved.

Strategy of surgical treatment of giant presacral tumor - a case report.

Presacral tumors are uncommon lesions that can be difficult to diagnose because of their nonspecific presenting signs and symptoms. Surgery is the mainstay of treatment as it establishes the diagnosis and prevents the adverse consequences associated with malignant degeneration and secondary bacterial infection. Large, highly vascularised pelvic tumors may pose intraoperative difficulties as bleeding and intraoperative tumor perforation. Cross-sectional imaging is essential in evaluating these lesions to determine the optimal surgical approach and the extent of resection. We emphasize a multidisciplinary expert individualized approach. We report a case of a presacral giant gastrointestinal tumor initially considered as unresectable but further on successfully managed by preoperative vascular embolization followed by resection via abdomino-perineal approach.

Plexiform fibrohistiocytic tumor: ultrastructural studies may aid in discrimination from cellular neurothekeoma.

Plexiform fibrohistiocytic tumor is a low-grade soft tissue malignancy that can at times be difficult to differentiate from the less biologically aggressive cellular neurothekeoma. The two entities, which may display identical clinical and histological features, cannot be distinguished by immunohistochemical or molecular diagnostic means. Electron microscopy may enable the accurate identification of problematic examples and thus aid in resolving these occasionally occurring diagnostic dilemmas. To illustrate typical variations in the ultrastructural appearance of plexiform fibrohistiocytic tumor, the authors present two diagnostically noncontroversial examples, and to demonstrate the potential diagnostic utility of electron microscopy in this setting, they present an example of plexiform fibrohistiocytic tumor that could not otherwise have been distinguished from cellular neurothekeoma.

Acquired mucosal indeterminate cell histiocytoma.

Indeterminate cell histiocytosis is an exceptional and controversial entity with variable clinical, histopathologic or immunohistochemical findings, sharing morphologic and immunophenotypic features from both Langerhans and non-Langerhans cell histiocytoses. Neoplastic cells express S-100 and CD1a antigens, but lack Birbeck granules. It has been reported in both adults and children, as solitary or multiple cutaneous lesions with rare extracutaneous involvement. We describe a 12-year-old boy with an indeterminate cell histiocytosis manifesting as a solitary verrucous papule on the mucosa of the glans penis. The morphologic features and diagnostic criteria of cutaneous indeterminate cell histiocytic proliferations are reviewed. The possible relationship between indeterminate cell and Langerhans cell histiocytoses is discussed.

Benefit of surgical treatment of lung metastasis in soft tissue sarcoma.

HYPOTHESIS: Patients with pulmonary metastatic soft tissue sarcoma benefit from resection, with long-term cure possible. DESIGN: Retrospective medical records review. SETTING: Academic tertiary care center. PATIENTS: Between January 1, 1991, and December 31, 2002, 61 patients (33 men and 28 women; median age at initial diagnosis, 42 years [age range, 18-74 years]) were surgically treated for pulmonary metastases of soft tissue sarcoma at University Hospital, Hamburg-Eppendorf, Germany. INTERVENTIONS: Sternotomy or anterior lateral thoracotomy was performed for metastasectomy, including wedge resection or lobectomy. MAIN OUTCOME MEASURE: The effects of clinical and pathologic factors on disease-specific survival were analyzed using the log rank test and a multivariate Cox proportional hazards model. RESULTS: Primary tumor size was pT1 in 13 patients and pT2 in 48 patients. The differentiation was high in 7 patients, intermediate in 19 patients, and low in 35 patients. The mean number of resected pulmonary metastatic lesions was 5 (range, 1-48). An anterolateral thoracotomy was performed in 39 patients, and sternotomy in 22 patients. There were no significant postoperative complications that required surgical revision. The perioperative mortality was 0%. At a mean follow-up of 60 months, the mean survival time after metastasectomy was 33 months (range, 2-125 months). The 5-year survival was 25%. The number of resected lung metastatic lesions had no prognostic relevance (P = .37). CONCLUSIONS: Patients with lung metastasis from soft tissue sarcomas benefit from surgical excision. This treatment has low complication rates and has a favorable influence on the course of the disease. Long-term survival is possible even when recurrent pulmonary disease is resected.

Use of a vascularized fibula bone flap and intercalary allograft for diaphyseal reconstruction after resection of primary extremity bone sarcomas.

BACKGROUND: The standard treatment for primary bone sarcomas of the extremities has become chemotherapy and limb salvage surgery. However, the difficulties in achieving reliable long-term healing with allograft reconstruction have led us to use vascularized fibula transfer to enhance healing. METHODS: From 1992 to 2003, 14 vascularized fibula transfers were performed at our institution for bone reconstruction in 12 patients with bone sarcoma. Free vascularized fibula transfers were performed in 13 cases, and a pedicled vascularized fibula transfer in one case. The mean age was 25 years (range, 6 to 71 years). Locations included the femur (n = 10), humerus (n = 1), and tibia (n = 3). The mean length of the vascularized fibula transfer was 17.4 cm (range, 10 to 24 cm). Indications for use of a vascularized fibula transfer included allograft nonunion (n = 8), and primary diaphyseal bone defect reconstruction combined with an intercalary allograft (n = 6). For all allograft nonunions, a vascularized fibula transfer was used with an onlay technique. For segmental bone defects, an intramedullary technique was used in three cases and an onlay technique in two cases. RESULTS: The overall mean time for bone union after a vascularized fibula transfer was 8.6 months (range, 3 to 24 months): 10 months (range, 5 to 24 months) for patients with allograft nonunions, and 6 months (range, 3 to 8 months) for patients who underwent immediate segmental bone reconstruction. All but one patient had successful bone union. One patient with persistent nonunion required a second vascularized fibula transfer. The mean time from initial limb salvage surgery to full use of the reconstructed limb without restrictions was 28 months (range, 13 to 45 months) for patients treated with a delayed vascularized fibula transfer for an allograft nonunion and 6 months (range, 3 to 8 months) for patients who underwent immediate reconstruction with a vascularized fibula transfer combined with an allograft. CONCLUSIONS: The use of a vascularized fibula transfer combined with an intercalary allograft to reconstruct bone defects after tumor resection can prevent allograft nonunion and result in decreased time to bone healing, leading to earlier patient recovery and return of function.

Pulmonary crystal-storing histiocytoma.

We describe the case of a 50-year-old woman with a lung tumor composed of crystal-storing histiocytes. These cells and associated plasma cells failed to show clonal light chain restriction, and the patient had no associated hematologic disorder. The differential diagnosis included crystal-storing histiocytosis, characterized by accumulation of crystallized immunoglobulins, a rare manifestation of monoclonal gammopathies/plasma cell dyscrasias. Crystal-laden histiocytes have previously been described in many organs. Four reports have described crystal-storing histiocytosis in the lung, always associated with a lymphoproliferative disorder. The present patient, 1 other case from our archive, and 1 case reported in the literature, all without an association with lymphoproliferative disorder, make a full description and definition of this lesion appropriate. The morphology, immunohistochemical profile, and electron microscopic features are described herein, and the term pulmonary crystal-storing histiocytoma is proposed. A practical algorithm is presented for the assessment of solitary lung masses composed of large histiocytic cells.