Giant-cell tumor of the patella with lung metastases: a case report

Giant-cell tumors are an infrequent clinical, radiological, and pathological entity observed in 5% of primary bone tumors. They generally occur at the epiphysis of long bones, particularly in the knee area but patellar localization seems very rare. Despite their perfectly benign histological aspect, giant-cell tumors may be aggressive, leading to local recurrence or even distant metastasis to the lung. We report a case of benign giant-cell tumor of the patella with lung metastasis observed in a 23-year-old woman. The aggressive radiological image was suggestive of chondrosarcoma. Histologically the differential diagnosis with chondroblastoma was difficult. The tumor and lung metastasis were treated by surgical resection. Four years later there has been no recurrence. We present the anatomic and clinical aspects of giant-cell tumor of the bone together with the diagnostic approach and the clinical course.     

Is radiotherapy important for low-grade soft tissue sarcoma of the extremity?

Radiotherapy and limb-preserving surgery has replaced amputation and compartmental resection for treatment of soft tissue sarcomas. However, the role of radiotherapy in low-grade tumors remains unclear. This study reviews the outcomes of 132 patients who received multimodality treatment for low-grade soft tissue sarcoma. Large primary tumors (> 5 cm) and the absence of radiotherapy correlated with local recurrence. Radiotherapy was most effective in patients operated on with marginal margins. Patients who were treated with wide surgical margins or had small tumors (< or =5 cm) showed no benefit with adjuvant radiotherapy. Size greater than 5 cm and local recurrence correlated with metastasis. Radiotherapy appears to be important in the management of low-grade soft tissue sarcoma. The principles of local treatment for low-grade soft tissue sarcoma should be the same as for high-grade tumors with a combination of surgery and adjuvant radiotherapy. In a subset of patients with small and widely excised tumors, consideration may be given to withholding radiotherapy. Local recurrence and metastasis from low-grade soft tissue sarcoma may occur as long as 1 decade after primary tumor resection. Long-term review of patients with low-grade tumors may be indicated.     

Clinico-pathological study of primary malignant chest wall tumors

Clinicopathological studies of primary malignant chest wall tumor on 16 cases operated between 1962 and 1988 were made. Of 9 osteogenic sarcomas, 8 cases were chondrosarcoma and 1 case was Ewing's sarcoma. Of 7 soft part sarcomas, 2 cases were fibrosarcoma, liposarcoma and neurogenic sarcoma respectively, and one case was hemangiosarcoma. Most of the cases had symptoms, such as chest mass and/or chest pain comprehend symptoms for more than one year. The intrathoracic growth of tumor is common, especially in osteogenic sarcoma. The maximum size of tumor was 8.2 cm in a mean diameter. Preoperative histological diagnosis is difficult to make even though various radiologic diagnosis or pathological technique as biopsy or cytology were assessed. And true rate of preoperative diagnosis is limited only 43.8%. Wide resection combined with the tissue distant more than 3 cm length from tumor is recommended and 6 cases underwent combined resection of diaphragm, pericardium or lung. 3 cases underwent chest wall reconstruction using the Marlex mesh and 10 cases were able to direct closure, in 13 cases with ribs resection. The 5 year survival rate of endurable cases was 62.2%, and that of soft tissue sarcoma (68.6%) is better than that of osteogenic sarcoma (41.7%). The recurrent or metastatic rate in high, 7 cases (43.8%), but reoperation was added for 5 cases of local recurrence or for a case of lung metastasis. 5 year survival rate of cases with recurrence or metastases is relatively good, 46.8%, especially excellent in 4 cases with recurred lesions after more than 2 years of tumor free interval.     

肩胛带骨肿瘤的外科治疗结果及评价

目的 探讨肩胛带骨肿瘤的手术切除方式、重建方法,观察术后功能恢复情况及临床结果.方法 回顾性分析1998年7月至2006年7月收治的71例肩胛骨周围骨肿瘤患者的病例资料,其中恶性肿瘤61例,骨巨细胞瘤10例.15例恶性肿瘤起源于肩胛骨,56例起源于肱骨近端.男42例,女29例;年龄11～62岁,平均36.5岁.手术方法:肩胛带离断术10例,单纯肩胛骨切除3例,肩胛骨切除、人工肩胛骨置换3例,部分肩胛骨及肱骨近端切除、假体置换8例,肱骨近端切除、假体置换47例.结果 10例骨巨细胞瘤患者肩周肌肉保留较好,术后MSTS功能评分平均28分.起源于肱骨近端的原发恶性骨肿瘤患者三角肌止点处均予以切除,术后肩外展30°～60°,MSTS功能评分平均23分.37例肱骨骨肉瘤患者中4例(10.8%)局部复发,2例骨转移,5例肺转移.7例转移患者均死亡.1例恶性骨巨细胞瘤患者出现肺转移死亡.3例尤文肉瘤患者出现肺转移死亡.5例肱骨及5例肩胛骨软骨肉瘤患者术后未见局部复发及转移.结论 肩胛带骨肿瘤切除、人工肱骨近端假体重建能保留完整肘部及手部功能、并发症少,是肩部恶性肿瘤的首选术式;肱骨近端骨肉瘤和下肢骨肉瘤比较预后较好;肱骨近端恶性肿瘤行关节内肿瘤切除和关节外肿瘤切除肿瘤的局部复发率接近,提示对多数肱骨近端恶性肿瘤可以采用关节内切除.     

Penile metastasis of chondrosarcoma of the jaw

Metastasis to the penis is an unusual event. Bladder and prostate tumors are the main sources of penile metastasis. Other sites include the rectosigmoid, kidney, and, less frequently, the pancreas, liver, nasopharynx, and lung. Other sources include malignant melanoma and Burkitt's lymphoma. The differential diagnosis includes idiopathic priapism, venereal or infectious disease, tuberculosis, Peyronie's disease, and primary penile tumor. Chondrosarcoma of the jaw is responsible for 10% of all chondrosarcomas that originate with craniofacial bones. Its behavior is usually characterized by local aggression; however, distant metastasis is uncommon. We report a case of chondrosarcoma of the jaw with penile metastasis. This is the first case described in published medical reports.     

Primary Sarcomas of the Larynx: A Clinicopathologic Study of 27 Cases

Primary sarcomas of the larynx are rare and are associated with diagnostic and treatment challenges. Studies of these tumors are limited, and most examples have been reported as small series. To further increase our understanding of laryngeal sarcomas, we reviewed our experience of an adult cohort. A retrospective search for laryngeal sarcomas from our pathology archives and consultation files of one of the authors was performed. We studied 27 primary laryngeal sarcomas that included 25 males, and 2 females, with a mean age of 60 years (range 33–85). The cases included conventional chondrosarcoma (16), well-differentiated liposarcoma (2), clear cell chondrosarcoma (1), leiomyosarcoma (2), high grade myxofibrosarcoma (2), high grade myofibroblastic sarcoma (1), low-grade myofibroblastic sarcoma (1), malignant granular cell tumor (1), and Kaposi sarcoma (1). Data on treatment and follow-up was available in 17 and 16 cases, respectively. 12 patients underwent partial laryngeal resection; five had total laryngectomy, and the patient with Kaposi sarcoma received combined highly active antiretroviral therapy and chemotherapy. Three patients developed local recurrence, and two patients developed metastases. The remaining patients with follow up had a favorable outcome and were disease-free after treatment. The important differential diagnosis of spindle cell sarcoma is sarcomatoid squamous cell carcinoma, and their distinction often requires extensive sampling of the mucosal surface and immunohistochemical analysis. The mainstay of treatment for laryngeal sarcomas is surgical removal, with the extent dictated by tumor type and grade. Adjuvant therapy is reserved for high-grade sarcomas and may be given in a neoadjuvant or adjuvant setting.     

Low Grade Fibromyxoid Sarcoma in Thigh

A low grade fibromyxoid sarcoma is a rare soft tissue tumor that has a tendency to develop in the deep soft tissue of young adults and the potential for local recurrence or distant metastasis. There have been several case reports and sporadic reports in the literature. However, only 1 case has been reported in Korea but without a follow-up result. We describe a 54-year-old female patient with a low-grade fibromyxoid sarcoma of the thigh that had been growing slowly for 34 years. A marginal resection of this tumor was performed. Currently, the patient is doing well without evidence of local recurrence or distant metastasis at 5 years after surgery.     

Sclerosing Rhabdomyosarcoma: Report of a Case Arising in the Head and Neck of an Adult and Review of the Literature

Sclerosing rhabdomyosarcoma is a unique rhabdomyosarcoma variant, characterized by a prominent hyalinizing matrix. A notable pitfall is the potential for the unusual matrix and often pseudovascular growth pattern of this lesion to lead to confusion with other sarcoma types, including osteosarcoma, chondrosarcoma, and angiosarcoma. Here we report a case of sclerosing rhabdomyosarcoma arising in a 40-year old male. The tumor was centered in the pterygomaxillary fossa with extensive infiltration into adjacent structures. Fine needle aspiration yielded a preliminary diagnosis of high-grade pleomorphic undifferentiated sarcoma, for which he received neoadjuvant chemotherapy and surgical resection. Microscopic examination showed a malignant spindled to round cell neoplasm with prominent osteoid-like, hyaline stroma. Focal rhabdomyoblastic differentiation and diffuse immunoreactivity for desmin and myogenin aided in diagnosis. Nineteen months status post primary resection, the patient expired with multiple lung and bony metastases. Among 39 cases reported thus far (including the present case), there is a broad age range (0.3–79 years), with an average age at presentation of 27 years. The most commonly involved sites are the extremities (n = 19) and head and neck (n = 15). Most cases have been treated by resection, often combined with radiation and/or chemotherapy. Out of 31 cases with follow-up information provided, 6 patients developed local recurrence, 7 patients developed regional or distant metastasis, and 5 patients died of disease. Herein we discuss the ongoing controversy regarding how sclerosing rhabdomyosarcoma might best fit into existing rhabdomyosarcoma classification schemes, based upon current clinicopathologic and molecular genetic evidence.     

Malignant fibrous histiocytoma in the right portion of the mandible with metastasis in pancreas

BackgroundMalignant fibrous histiocytoma is a sarcoma of uncertain origin that can be found both in soft tissues and in bones. It is currently called undifferentiated pleomorphic sarcoma not otherwise specified and it represents a final common pathway in several tumors that are subject to the progress of dedifferentiation.Local recurrence of the tumor in the same location where it was originated occurs in 20–30% of the total number of soft tissue sarcomas. It is less frequent in limbs and more likely to recur in retroperitoneal sarcomas and in head and neck.Most tumors grow during the first two years after treatment.Most sarcoma metastases, especially those in the head and neck (including malignant fibrous histiocytoma) present as a lung disease (90%). Extrapulmonary diseases are not frequent and can occur in lymph nodes (10%), bones (8%) and in the liver (1%).Case report61 years old woman with history of malignant fibrous histiocytoma in the right portion of the mandible, resected four years ago. Subsequent checkups did not show any disease. The patient reports a constant abdominal pain not diagnosed, related to a pancreatic nodule.ConclusionMalignant fibrous histiocytoma is a potentially curable disease. The most important part of the treatment is complete surgical removal, usually followed by adjuvant radiation therapy. Chemotherapy is a treatment usually used only on those patients with higher recurrence risk or on those showingrecurrence or who have already showed recurrence.     

A case of carcinosarcoma of the urinary bladder

A 66-year-old woman visited our hospital complaining of painful, irritative urinary symptoms and macroscopic hematuria. Cystoscopy revealed a non-papillary tumor covered with necrotic tissue on the right side of the posterior wall of the bladder. Transurethral resection was performed ; histologically, the tumor was found to be composed of carcinomatous and sarcomatous elements. The carcinomatous element consisted of urothelial and squamous cell carcinomas. The sarcomatous element was composed of osteosarcoma, chondrosarcoma and spindle cell sarcoma. Immunohistochemical examination showed that the carcinomatous component was positive for cytokeratin and the sarcomatous component was positive for S-100 protein. The patient underwent total cystectomy with ileal conduit under the diagnosis of carcinosarcoma. Pathological examination showed no residual tumor. She was followed up with no signs of recurrence or metastasis. Computed tomography (CT) at nine months following surgery showed no evidence of recurrence. However, thirteen months after the operation, she complained of lower abdominal pain, and CT demonstrated a bulky intrapelvic tumor and right hydronephrosis. Her condition worsened rapidly and she died one month later.     

累及骶骨的骨盆恶性肿瘤切除及重建

目的 探讨累及骶骨的骨盆恶性肿瘤合理的手术切除及重建方式.方法 1999年7月至2007年7月,共有19例累及骶骨的骨盆恶性肿瘤患者在北京大学人民医院骨肿瘤科接受肿瘤切除重建手术.平均年龄37岁(12～78岁);男性12例,女性7例. 其中,软骨肉瘤5例、尤文肉瘤4例、骨肉瘤4例、恶性纤维组织细胞瘤(MFH)1例、骨巨细胞瘤1例、转移癌4例.切除髂骨翼及部分骶骨、保留髋臼手术10例,采用了钉棒系统内固定,其中5例患者同时进行了自体腓骨或髂骨植骨;切除部分骶骨、髂骨翼及髋臼手术9例,应用组配式人工半骨盆重建骨盆环完整性.结果 肿瘤学结果:7例出现局部复发(7/19,36.9%),其中骨肉瘤2例、软骨肉瘤2例、尤文肉瘤2例及转移癌1例.19例患者随访时间为1至7年,平均4.5年.功能结果:9例保留髋臼、钉棒重建的患者术后正常行走,无步态异常.9例行Ⅱ区肿瘤切除、人工半骨盆重建的患者中,8例术后2个月能够扶拐行走.ISOLS评分平均20分以上,其中,良好3例,一般5例,较差1例.人工半骨盆重建的患者中,术后脱位1例,行切开复位;因深部感染取出假体1例.结论 对于保留髋臼的髂骨肿瘤切除,采用钉棒内固定结合自体骨植骨是一种理想的重建骨盆环稳定性的方法,可使患者早期恢复行走功能.将股骨头颈植于骶骨侧方,将组配式人工半骨盆卡于质骨块的下方,重建累及骶骨及髋臼的骨盆切除,是一种可取的重建方式.     

“Dedifferentiation” and High-Grade Transformation in Salivary Gland Carcinomas

“Dedifferentiation” and/or high-grade transformation (HGT) has been described in a variety of salivary gland carcinomas, including acinic cell carcinoma, adenoid cystic carcinoma, epithelial-myoepithelial carcinoma, polymorphous low-grade adenocarcinoma, myoepithelial carcinoma, low-grade mucoepidermoid carcinoma and hyalinizing clear cell carcinoma, although the phenomenon is a rare event. Recent authors tend to preferably use the term HGT instead of “dedifferentiation” in these cases. HGT-tumors are composed of conventional carcinomas juxtaposed with areas of HG morphology, usually either poorly differentiated adenocarcinoma or “undifferentiated” carcinoma, in which the original line of differentiation is no longer evident. The HG component is generally composed of solid nests, sometimes occurring in cribriform pattern of anaplastic cells with large vesicular pleomorphic nuclei, prominent nucleoli and abundant cytoplasm. Frequent mitoses and extensive necrosis is evident. The Ki-67 labeling index is consistently higher in the HG component. p53 abnormalities have been demonstrated in the transformed component in a few examples, but the frequency varies by the histologic type. HER-2/neu overexpression and/or gene amplification is considerably exceptional. The molecular-genetic mechanisms responsible for the pathway of HGT in salivary gland carcinomas largely still remain to be elucidated. Salivary gland carcinomas with HGT have been shown to be more aggressive than conventional carcinomas with a poorer prognosis, accompanied by higher local recurrence rate and propensity for cervical lymph node metastasis, suggesting the need for wider resection and neck dissection.     

Low-grade central chondrosarcoma: difficult diagnosis in an adolescent girl

Central chondrosarcoma of the tibia is exceptional, particularly in young patients. Low-grade tumors raise difficult problems for histological distinction with enchondroma. We report a case of grade 1 chondrosarcoma located in the upper portion of the tibia in a 17-year-old girl. After radical surgery, outcome was favorable with no recurrence or metastasis at three years follow-up. The distinction between low-grade central chondrosarcoma and enchondroma is one of the most difficult challenges in bone pathology. Clinical, radiographic and pathological data must be considered together to reach certain diagnosis.     

Three cases of pulmonary hamartoma appearing after radical nephrectomy for renal cell carcinoma

We report 3 patients with pulmonary hamartoma, all of whom had undergone nephrectomy for renal cell carcinoma. A lung tumor was detected 2 to 9-months following nephrectomy. Preoperative diagnosis was pulmonary metastasis from renal cell carcinoma and pulmonary tumor resection was performed in each case. There was a 9- to 12-month interval between the detection and resection of the lung tumor. The histological diagnosis of the lung tumor in all three patient was pulmonary hamartoma. Following the resection of the lung tumor, recurrence was not noted in any of the patients.     

Malignant tumours of bone: clinical aspects and natural course.

An accurate pathological diagnosis must be made prior to treatment of a primary malignant bone tumour. Consideration must be given to the clinical and radiologic aspects as well as the histology. Both benign and malignant tumours occur more frequently in certain decades. A search should be made for precursor lesions such as Paget's disease. The presenting manifestations of pain, a mass and dysfunction are not specific for tumours. Laboratory tests may be helpful, especially in distinguishing tumours from infections and metabolic diseases. Metastasis is usually via the blood stream to the lungs and bones. The low survival rate following amputation for osteosarcoma and radiation therapy for Ewing's sarcoma has been improved by chemotherapy. The lower-grade tumours such as aggressive giant cell tumour and low-grade chondrosarcoma can often by treated successfully by resection and insertion of an autograft, an allograft or a metallic implant.     

Dedifferentiated chondrosarcoma of the cervical spine in a patient with multiple osteochondromas

Dedifferentiated chondrosarcoma (DCS) is a biphasic tumor, comprising a low-grade chondrosarcoma juxtaposed to an anaplastic sarcoma with a high degree of malignancy. DCS of the spine arising from osteochondromas is extremely rare. We report a very unusual case of a 28-year-old female with DCS originating from a cervical spine osteochondroma. At the age of 12 the patient was diagnosed as having multiple osteochondromas. At the age of 28, she consulted our clinic complaining of rapid increase of the mass and dull pain. Radiological examination revealed a 12 cm × 8 cm sized mass with irregular calcification and cauliflower-like surface, arising from the second to the sixth cervical spines. The tumor was removed with the suspicion of secondary chondrosarcoma arising from the predisposing osteochondroma of the spine. Histological examination revealed the tumor was composed of a biphasic pattern, one with well differentiated chondrosarcoma and the other with high grade spindle cell sarcoma (malignant fibrous histiocytoma). The tumor was diagnosed as DCS in the spine. The patient died of pulmonary metastasis at 6 years after surgery.     

A rare case of acral myxoinflammatory fibroblastic sarcoma at the hand

One case of acral myxoinflammatory fibroblastic sarcoma is presented. Most of the soft tissue tumefactions of the distal extremities are benign. Sarcomas are rare and usually high grade. Acral myxoinflammatory fibroblastic sarcoma is a unique low-grade tumor described by Kindblom in 1998. This neoplasm has a propensity for digits, presents a low rate of metastasis and is characterized by a high rate of local multiple and aggressive recurrence. The clinical presentation, the frequent association with tenosynovitis and the extensive inflammatory infiltrate can lead to misdiagnosis by both the surgeon and the pathologist. Correct and early diagnosis depends on a good knowledge of histological and immunohistochemical findings. It is important to avoid recurrences recurring to an immediate wide excision of the tumor.     

Long-term follow-up of cartilaginous tumors of the larynx.

BACKGROUND: Cartilaginous tumors of the larynx are uncommon. A literature review disclosed approximately 250 cases since 1816; the cricoid cartilage is the most common site. The rarity of these tumors has made for limited experience and, as a consequence, our knowledge is incomplete. OBJECTIVE: To report surgical results as well as long-term follow-up on 6 patients with cartilaginous tumors of the larynx. DESIGN: A 28-year retrospective study with the patients followed-up from 6 to 28 years (average, 17.8 years). METHODS: Six adult white male patients with cartilaginous tumors of the larynx: 4 low-grade chondrosarcoma (1 of the thyroid and 3 of the cricoid) and 2 chondroma of the cricoid. Surgical treatment included total laryngectomy of the thyroid and 1 of the cricoid chondrosarcoma, and conservation surgery of the other 4 cricoid tumors: the 2 patients with chondrosarcoma had total resection of the cricoid cartilage with thyrotracheal anastomosis, and the 2 patients with chondroma had local tumor resection using a laryngofissure approach. RESULTS: The margins of the specimen were negative for tumor in the 6 patients. On follow-up, none of the patients had regional or distant metastasis or tumor-related death. One of the patients with cricoid chondrosarcoma developed recurrence 8 years after conservation surgery, and required a total laryngectomy for salvage. Survival rate tumor-free at 5 years was 100% and at 10 years 67%, co-morbidity being responsible for the decrease in survival rate. CONCLUSIONS: Based on this small series of patients, the long-term follow-up of benign and low-grade malignant tumors suggests that the surgical approach and prognosis does not depend on histologic distinction and, importantly, underdiagnosed malignancy on tumor sampling and recurrent chondrosarcoma, managed with salvage surgery, have no adverse impact on patient survival. Total resection of the cricoid cartilage with thyrotracheal anastomosis over a stent proved an alternative surgical technique in chondrosarcoma who otherwise would have been treated by total laryngectomy.     

Low grade fibromyxoid sarcoma (Evans tumour) of the arm

A low-grade fibromyxoid sarcoma is a rare soft tissue tumour that has a tendency to develop in the deep soft tissue of young adults with potential for local recurrence or distant metastasis. We describe a 40-year-old female patient with a low-grade fibromyxoid sarcoma of the shoulder that had been excised twice in the past and then had recurred after a few months. A wide resection of this tumour with flap reconstruction was performed followed by radiation to the area. The patient had no evidence of local recurrence or distant metastasis at 2 years after surgery.KEY WORDS: Evans tumour, low-grade fibromyxoid sarcoma, shoulder     

Bladder metastasis of renal cell carcinoma : report of a case

A 65-year-old man presented with gross hematuria in 2004. Computed tomography (CT) showed a left renal mass, and he underwent laparoscopic radical nephrectomy. Pathological diagnosis was clear cell carcinoma (pT2N0M0, G2＞G3). Four years later, a right adrenal tumor was disclosed by follow-up CT. Then laparoscopic adrenectomy was performed. Histology showed metastasis of the renal clear cell carcinoma. In 2009, he noticed gross hematuria, and cystoscopy revealed a 2cm solitary, non-papillary tumor at the anterior wall of the bladder. At the same time, small solitary liver metastasis (6 mm) was observed on abdominal CT. Transurethral resection of the bladder tumor and resection of liver tumor was performed, and pathological diagnosis was clear cell carcinoma both in vesical and hepatic masses. Nine months after the last surgery, he is living with no obvious tumor recurrence. To our knowledge this case is the 34th case of bladder metastasis from renal cell carcinoma in the Japanese literature. We reviewed literature and discuss the clinical features of bladder metastasis of renal cell carcinoma.