Clear cell sarcoma arising from the chest wall: a case report

Clear cell sarcoma is a rare malignant soft tissue neoplasm that usually arises adjacent to tendons or aponeuroses. The clinical course is rather slow, with repeated local recurrences followed by late metastases and eventual death. The principal sites of this neoplasm are the extremities, but tumors do occur in the trunk on rare occasions. We report a case of clear cell sarcoma arising from the chest wall. The patient, a 20-year-old woman, had noticed a chest wall mass and pain for 2 years. Biopsy of the mass showed abundant nests of round cells with clear cytoplasm. On immunohistochemical examination, tumor cells were strongly immunoreactive for S-100 and HMB-45. A diagnosis of clear cell sarcoma was confirmed. There was no other lesion found in the patient through routine imaging studies. She was treated with two courses of chemotherapy using ifosfamide, carboplatin, and etoposide. Subsequently, the tumor, including adjacent tissue, the chest wall, and sternum, was resected with a wide margin; and the defect of the chest wall was covered with Marlex mesh fabric, regin, and a musculocutaneous flap. She has shown no symptoms or signs of recurrence during 2 years of follow-up.     

A multidisciplinary approach to giant soft tissue sarcoma of the chest wall: A case report

IntroductionSoft tissue sarcomas of the chest wall are exceptionally rare entities that present as painless slow growing masses. Resection is often precarious due to involvement of vital structures, and patients are left with large chest wall defects postoperatively requiring extensive reconstruction.Presentation of caseWe present a case report of a 29 year-old man who presented with a giant soft tissue sarcoma of the chest that had been growing slowly for one year prior to presentation. The patient had a biopsy that was positive for sarcoma, and PET CT demonstrated a large lobulated mass in the left chest wall with an SUV of 6.7. He received 50 Gy of radiation therapy; however, the mass continued to grow in size. He subsequently underwent an en-bloc resection of the mass with latissimus and serratus muscle primary reconstruction. Final pathology showed a 27 cm high-grade fibrosarcoma with prominent myxoid component. To our knowledge, this is the largest soft tissue sarcoma of the chest wall reported in the literature. Postoperatively, the patient received 6 cycles of adjuvant chemotherapy.DiscussionSurgery is the mainstay of treatment, and chemotherapy and radiation are used in specific circumstances. Risk of recurrence is dependent on many factors, including histologic subtype, grade, and size of tumor. Long term surveillance with physical exam and imaging is recommended.ConclusionWe feel that the multidisciplinary approach is crucial for optimal management of large soft tissue sarcomas. We recommend this approach to all patients with chest wall sarcomas.     

Chondrosarcoma of the chest wall: a clinical analysis

Purpose. To discuss the management of different histological types of chondrosarcoma (CS) in the chest wall based on our clinical experience. Methods. The subjects of this study were 16 patients with CS of the chest wall surgically treated by resection at our institute between September 1981 and August 2000. There were 11 men and 5 women ranging in age from 23 to 74 years. The median follow-up period was 54 months. The tumor was located only in the ribs in ten patients, in the sternum and ribs in three, only in the sternum in two, and in the ribs and spine in one. The surgical margins were wide in 12 patients and marginal in four. Reconstruction using Marlex mesh combined with moldable metal plates was carried out to prevent flail chest in nine patients, resection alone was performed in five patients, and a muscular flap was used in two patients. Results. The survival rate was 86% after a median follow-up period of 54 months. One operative death (6.2%) occurred, and another required temporary tracheostomy. There were no infections in this series. Oncological outcome was clinically related to surgical margins and recurrence. The postoperative respiratory function test result was 10% less than the preoperative one. Conclusion. This series demonstrated that wide resection is the treatment of choice for chest wall CS and that Marlex mesh combined with metallic mouldable plates is a reliable technique for reconstruction. Received: April 3, 2001 / Accepted: November 20, 2001     

Primary hydatid cyst of the rib mimicking chest wall tumor: a case report

Hydatidosis, caused by Echinococcus granulosus, is still an endemic parasitic disease in the Mediterranean area, South America, North Africa, and Australia. The most common anatomic location of hydatid disease is the liver, followed by the lung. Hydatidosis is located in the bones in 0.5% to 2% of all cases. A hydatid cyst can be seen in any part of the body. However, primary hydatid cyst of the rib in childhood is exceedingly rare. We report the first case of a pediatric hydatid cyst of the rib mimicking chest wall tumor.     

Unilateral multifocal mesenchymal hamartoma of the chest wall: a case report and review of literature

We report a case of unilateral multifocal mesenchymal hamartoma of the chest wall diagnosed on radiologic imaging, which was proven at surgery and histopathology. To the best of our knowledge, only 3 cases of unilateral multifocal mesenchymal hamartoma of the chest wall have been reported previously in literature in children.     

A new method of stabilizing the chest wall following major chest wall resection

Summary Prosthetic materials, such as metals, marlex mesh and methyl methacrylate have been used for stabilization of the chest wall after resection of large areas of rib cage. Such materials are contraindicated in an infected area. A new method of providing a stable chest wall using autogenous tissue is presented.     

Reconstruction of the chest wall following extended interscapulothoracic amputation and chest wall resection

Summary Extended interscapulothoracic amputation is a major operative procedure indicated in the treatment of malignant primary bony and soft tissue tumors involving the shoulder girdle and chest wall. The technique of chest wall resection and its reconstruction is described in two patients with recurrent malignant fibrous histiocytoma following extended interscapulothoracic amputation some months earlier. The stability of the chest wall was restored by using marlex mesh as a sandwich of two layers of mesh with methylmethacrylate interposed. Because of damage of the tissue around the chest wall resection by previous radiation therapy, free myocutaneous flaps were used for closure of the defects. Using this technique for reconstruction of large areas of the chest wall, it is feasible to restore sufficient pulmonary function and to obtain closure under unfavorable conditions. This operative technique can be used as a curative or palliative treatment following interscapulothoracic amputation of recurrent musculoskeletal tumors.     

The impact of surgical excision in chest wall rhabdomyosarcoma: a report from the Children's Oncology Group

Aims

Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood. Patient age, size, histologic finding, and site of the tumor are primary determinants of prognosis in RMS. Chest wall RMS is a site in which the limitations of surgical excision are realized. We aim to determine the impact of surgical excision in chest wall RMS.

Methods

A retrospective chart review was conducted of all 130 pediatric patients enrolled in the Intergroup Rhabdomyosarcoma Study (IRS) with chest wall rhabdomyosarcoma from the first (I) through fourth (IV) IRS with follow-up to June 2005. Median follow-up was 12.1 years (4.6-27.2 years).

Results

There was a significant improvement in failure-free survival (FFS) and overall survival (OS) between the first IRS study, I, and IRS-IV. The estimated FFS and OS at 5 years in IRS I was 30% and 40%, respectively, compared to 68% and 78%, respectively, in IRS-IV (P = .03 and P = .05, respectively). There was no association between histologic finding or size and FFS or OS. However, all patients who presented without metastasis had an FFS and OS of 49% and 61%, respectively, compared with metastatic patients, 7% and 7%, respectively (P < .001). Five-year FFS of group I, II, and III patients was 52%, 52%, and 45%, respectively, and OS was 65%, 60%, and 59%, respectively. There was no significant difference in 5-year FFS or OS in patients who had a complete resection (group I), complete resection with positive microscopic margins (group II), or biopsy or partial resection only (group III). In groups I to III patients, the local and regional failure rate at 5 years is 25% and 6%, respectively.

Conclusions

The most significant impact on outcome in chest wall RMS patients is metastatic disease at diagnosis. The locoregional failure rate is high but does not appear to impact survival. Alternative treatment strategies are needed for chest wall RMS, but aggressive surgical excision may not be necessary.     

Hodgkin lymphoma presenting with chest wall involvement: a case series

Chest wall tumors in the pediatric population can have a variety of etiologies, malignancy being the most worrisome. Hodgkin lymphoma (HL) rarely presents as a chest wall mass in the pediatric population. In this report, we describe 3 male pediatric patients, all of whom had chest wall masses present at the initial diagnosis of HL. We also discuss the literature on this topic. We conclude that malignancy and, more specifically, HL should always be considered when evaluating a pediatric patient who presents with a chest wall mass.     

Efficacy and safety of continuous low-irradiance photodynamic therapy in the treatment of chest wall progression of breast cancer

Background

Photodynamic therapy (PDT) is a binary therapy using a drug and high-energy light source. PDT is approved for several premalignant and malignant conditions. Recent in-vitro and animal data suggest that enhanced tumor-specific cytotoxicity can be achieved with far less collateral damage to normal surrounding tissues if PDT is administered continuously at a lower dose rate for extended periods of time. Based on these promising preclinical data, we conducted a Phase I clinical trial of continuous low-irradiance photodynamic therapy (CLIPT) using 630 nm laser energy and intravenously administered porforin sodium as the photosensitizer. We determined the maximum tolerated dose (MTD) of CLIPT on skin and tumor response in subjects with cutaneous and subcutaneous metastatic nodules who had failed radiation and surgery.

Methods

Patients with cutaneous and/or subcutaneous metastatic nodules that had failed radiation and surgery were offered enrollment into the trial. The initial study design planned for sequential cohorts of six subjects to be treated at increasing laser intensity, starting at 100 J/cm² administered continuously over 24 h (10⁻² dose rate compared with standard PDT). Dose-limiting toxicity was defined as partial or full-thickness necrosis of the surrounding tumor-free, previously irradiated skin. The MTD was defined as the highest laser energy at which ≤33% of subjects experienced the dose-limiting toxicity. Subjects received intravenous porfirmer sodium 0.8 mg/kg 48 h before commencing CLIPT. Response rates and quality of life measures were assessed.

Results

Nine subjects were enrolled with chest wall progression of breast cancer following mastectomy. All had failed prior surgery and electron-beam radiation therapy. The initial two subjects were treated at 100 J/cm² and developed partial thickness skin necrosis. Dose reduction was therefore instituted, and the next cohort was treated at 50 J/cm². None of the subsequent seven subjects suffered partial or full thickness necrosis, thus establishing the MTD at 50 J/cm² over 24 h (0.5 mW irradiance). Six of the nine subjects (67%) had either a complete or partial clinical response. Of note, two subjects had significant regression of tumor nodules distant from the treatment field. Of the eight subjects whose terminal deoxynucleotidyl transferase dUTP nick end labeling assay results were available, 8 (100%) demonstrated histologic response to treatment as evidenced by either tumor apoptosis or regression. Quality of life measures were improved following treatment—particularly bleeding and pain from the tumor nodules.

Conclusions

The MTD of CLIPT was established at 50 J/cm² administered continuously over 24 h. These preliminary data suggest CLIPT may be an effective, low-morbidity therapeutic modality in the treatment of cutaneous and subcutaneous metastases of breast cancer following mastectomy. Further evaluation in a larger cohort is warranted to better assess efficacy and optimize the intervention.     

Chondromyxoid fibroma of the chest wall

A chondromyxoid fibroma of the chest wall is a rare occurrence. A case study of a 40-year-old woman diagnosed with this tumor in the left chest wall is presented. The lesion was removed by video-assisted thoracic surgery (VATS), and the final pathology examination confirmed a chondromyxoid fibroma. At present, this patient has continued to visit our outpatient unit regularly and has shown no recurrence during the past 1 year.     

Osteosarcoma of the rib: A challenge of diagnosis and surgical intervention: A case report

Introduction and importanceChest wall tumour could grow from various tissues composing the chest wall. Diagnosis and treatment of patients with chest wall tumour pose several challenges.Case presentationWe present a case of 55 year old woman with chief complaint of a painfull mass on her chest wall. Patient was diagnosed with osteosarcoma of the ribs. Patient underwent surgery and was hospitalized for seven days before discharged.Clinical discussionOsteosarcoma of the ribs is a rare case with wide resection as the main treatment. In this case, chest wall reconstruction was needed to prevent impaired chest wall mechanical function in respiration.ConclusionDiagnosis and treatment of osteosarcoma of the ribs pose challenges. The main treatment of the osteosarcoma of the ribs is wide resection, followed by reconstruction of chest wall when needed. Reconstruction could be performed with autologous tissue, gore-tex, and mesh. The adjuvant chemotherapy was considered to give for better outcome.     

Chest wall mesenchymal hamartoma associated with a massive fetal pleural effusion: a case report

We report on an extremely rare chest wall mesenchymal hamartoma associated with a massive fetal pleural effusion. Prenatal ultrasound examination demonstrated a heterogeneous mass in the right thorax associated with a massive pleural effusion and right lung compression at 29 weeks of gestation. The patient underwent pleuroamniotic shunting at 30 weeks and was delivered at 33 weeks by cesarean delivery secondary to fetal distress. After management of the respiratory distress and evaluation of the mass, surgery was performed at day of life 8. Histological examination confirmed the diagnosis of a chest wall mesenchymal hamartoma.     

Radiation-associated malignant tumors of the chest wall

Background: Malignant postirradiation cancers of the chest wall are uncommon, and data concerning results of treatment are sparse. We assessed patient characteristics as well as prognostic factors of these tumors compared with those arising de novo and analyzed treatment results of both groups. Methods: Records of 361 patients with primary malignant tumors of the chest wall admitted to our institution between 1949 and 1989 were reviewed retrospectively. Previous radiotherapy to the site of the tumor was noted with latency period and dose. Survival was calculated via the Kaplan-Meier method, and comparisons of survival were made by log-rank analysis. Results: In 351 patients with primary malignant tumors of the chest wall, 21 lesions (6%) arose in an irradiated field. Eighty-eight patients had chondrosarcoma (age range 5–86 years, median 49; male:female [M:F] ratio 1.3:1), two cases of which arose in an irradiated field; 38 patients had osteosarcoma (age range 11–78 years, median 42; M:F ratio 1.5:1), 11 cases (29%) of which arose in an irradiated field; 149 patients had soft-tissue sarcoma (age range 1–86 years, median 38; M:F ratio 2:1) seven cases (5%) of which arose in an irradiated field; 52 patients had Ewing's sarcoma (age range 2–39 years, median 16; M:F ratio 1.6:1) no cases of which arose in an irradiated field; and 24 patients had a solitary plasmacytoma (age range 37–75 years, median 59; M:F ratio 2.4:1) one case (5%) of which arose in an irradiated field. Prior radiotherapy had been performed for Hodgkin's disease (n=8), breast cancer (n=5), and various other indications (n=8). The maximum radiation dose administered ranged from 1,250 to 9,500 cGy (median 4,140). The latency period from previous irradiation to diagnosis ranged from 2 to 19 years (median 7). The primary therapy of all radiation-associated tumors was resection, except for three patients. There was no significant difference in survival between those malignant chest wall tumors arising in an irradiated field compared with those arising de novo. Conclusions: Twenty-nine percent of patients with primary osteosarcoma and 2–5% of patients with primary chondrosarcoma, soft-tissue sarcoma, or plasmacytoma of the chest wall seen at this institution have a tumor arising in the field of prior irradiation. Because the outcome after operative therapy appears to be similar, these patients should be offered identical treatment to those whose tumors arise de novo.Presented at the 48th Annual Cancer Symposium of The Society of Surgical Oncology, March 23–26, 1995, Boston, Massachusetts.     

Autologous rib transplantation and terylene patch for repair of chest wall defect in a girl with Poland syndrome: a case report

Poland syndrome is a constellation of anomalies including absent pectoralis muscle and rib defects, even with malformations of the upper extremity that more often affects boys than girls. This case describes a 5-year-old and 4-month-old girl diagnosed with Poland syndrome that required chest wall reconstruction. An autologous rib transplantation and terylene patch were used for repair of the chest wall defect. The girl had a satisfactory postoperative recovery. This technique was an effective method of treatment of this condition.     

Combined therapy for malignant tumors of the chest wall in children.

Of 1168 children with malignant solid tumors who were admitted to St. Jude Hospital between 1962 and 1976, 22 (1.8%) had primary malignancies of the chest wall, excluding the mediastinum. The treatment plan for these patients varied with the resectability of the tumor, but always included chemotherapy with or without irradiation. Six children had totally resectable tumors and another two had initially unresectable tumors converted to a resectable state with preoperative therapy. All 8 patients have been disease-free for 3–90 mo (median 18.5). Of 11 children with unresectable disease, 4 remain disease-free for 7–175 mo following chemotherapy and irradiation only (3 of 9) or both modalities plus partial resection (1 of 2). The 3 remaining children received en bloc resections for recurrent rhabdomyosarcoma following chemotherapy and irradiation: 2 died at 15 and 18 mo after diagnosis and 1 remains disease-free for 13 mo.The availability of effective combined therapy methods for local control of primary tumors and prevention of metastases made possible a more conservative surgical approach for 4 of 8 patients with resectable disease and permitted conversion of initially unresectable tumors to a resectable state in 2 patients. Moreover, these measures resulted in decreased morbidity without apparent compromise of tumor-free survival. Overall, 13 of 22 patients survive without evidence of disease: 3 of 3 patients with neuroblastoma, 4 of 6 with rhabdomyosarcoma, 4 of 10 with Ewing's sarcoma, 1 of 2 with osteosarcoma, and 1 patient with malignant histiocytoma.     

Spontaneous abdominal wall endometrioma: A case report

IntroductionThe abdominal wall is the commonest site of extra pelvic endometriosis (endometrioma), defined as the presence of functional endometrial gland and stroma outside the uterine cavity. Spontaneous abdominal wall endometriosis (AWE) represents an ectopic functional endometrial tissue situated superficial to peritoneum in a scar less abdomen. Spontaneous AWE is rare, accounting for 20% of all abdominal wall endometriosis. It is unfamiliar to general surgeons because of a large number of potential pitfalls in its diagnosis.Clinical presentation and interventionWe report a case of spontaneous abdominal wall endometriosis (endometrioma) presenting to our general surgery clinic with a painful nodular mass in hypogastric area below umbilicus. The initial diagnosis was a desmoid tumour of the anterior abdominal wall. Pain was a remarkable complaint in our patient. Abdominal wall endometrioma was diagnosed by histopathology postoperatively. Excision planned, during operation, one mass was spotted and excised within healthy limits. Histopathology revealed: endometrial glands surrounded by mantle of endometrial stroma, few scattered hemosiderin laden macrophages and fibrous scar tissues. Our patient had no previous scar. She was discharged from hospital on 5th postoperative day uneventfully. (On account of uncontrolled DM and Hypertension). One year of follow up after the surgery, she is free from disease and no recurrence has been observed.DiscussionEndometriosis is characterised by the presence of endometrial glands and stroma outside the uterine cavity with the maximum prevalence reported in the 4th decade of life. Most cases of spontaneous endometriosis occur in a scar less abdomen. Multiple imaging techniques have been used and described for its diagnosis. The alleged aetiopathogenesis of spontaneous endometrioma is still debatable. It is usually diagnosed by the histopathology and the preferential treatment in cases of endometrioma is total excision of the mass. The hormonal therapy can be added to surgical excision if there is proven pelvic endometriosis. The surgical excision should be wide enough to prevent its recurrence.ConclusionSpontaneous abdominal wall endometriosis is an extremely rare gynaecological entity, accounts for 18–20% of all abdominal wall endometriosis. The diagnosis of abdominal wall endometrioma is hardly established prior to surgery. The triad: mass, pain and cyclic symptom aids in diagnosis, unfortunately cyclic symptom is not present in all cases (as in our case). Spontaneous abdominal wall endometrioma is usually diagnosed by high index of clinical suspicion and histopathology. The results of imaging techniques are nonspecific. It may pose a diagnostic dilemma due to its rarity and atypical presentation. The preferential treatment of choice is wide excision.     

Perinatal evolution of mesenchymal hamartoma of the chest wall

Mesenchymal hamartoma of the chest wall (MHCW) is a rare condition. Previously, surgical resection has been advocated with considerable post-operative morbidity. Evidence for conservative management is lacking because the natural history of MHCW is unknown. We present serial measurements of an antenatally detected MHCW (8 antenatal ultrasounds and 2 postnatal computed tomographic scans). The study demonstrates that the relative tumor size peaked at birth and then decreased postnatally. Based on this evidence, we believe that MHCW can be managed conservatively in an asymptomatic patient.     

Pedicled full‐thickness abdominal flap combined with skin grafting for the reconstruction of anterior chest wall defect following major electrical burn

Successful reconstruction of extensive anterior chest wall defect following major electrical burn represents a very challenging surgery. Herein we report the first case using pedicled full‐thickness abdominal flap combined with skin grafting to treat this injury with severe infection and exposure of pericardium and ribs in a Chinese patient. Following the performance of chest debridement to remove necrotic and infected tissues and the injection of broad‐spectrum antibiotics to reduce infection, a pedicled full‐thickness abdominal flap was used to cover the exposed pericardium and ribs, and skin grafting from the right leg of the patient was done to cover the exposed vital tissues. The patient was followed up for a total of 3·5 years, and satisfactory cosmetic and functional outcomes were obtained without complications. This report provides an effective method for the surgeons who encounter similar cases where reconstruction of extensive anterior chest wall is required.