Hemangiopericytoma/solitary fibrous tumor of pectoralis major muscle mimicking a breast mass

INTRODUCTIONHemangiopericytoma (HPC)/solitary fibrous tumor (SFT) is a very uncommon tumor of uncertain malignant behavior. In 1942, Stout and Murray first characterized these neoplasms as “vascular tumors arising from Zimmerman's pericytes” and till now hemangiopericytomas and solitary fibrous tumors of the soft tissues are regarded as features of the same entity in the soft tissue fascicle.PRESENTATION OF CASEWe present a case of hemangiopericytoma/solitary fibrous tumor of the pectoralis major muscle in a 33-year-old female. She first noticed a painless mass in her right breast. Ultrasound of the breast revealed a large heterogeneously hypoechoic lesion within the pectoralis major muscle. Fine needle aspiration of the tumor did not produce any meaningful result. The lesion was completely removed by surgical resection. Histologically, the tumor had staghorn-like vasculature and immunohistochemistry for CD34 was positive, whereas desmin, smooth-muscle actin, S-100 protein, cytokeratins (AE1/AE3) and epithelial membrane antigen (EMA) were all negative. A diagnosis of hemangiopericytoma/solitary fibrous tumor was rendered.DISCUSSIONTumors comprising the HPC/SFT spectrum represent a small subset of soft tissue sarcomas and are found virtually at any site in the body. Wide surgical resection can achieve favorable long-term survival.CONCLUSIONDue to the rarity and unpredictable biological potential of these tumors, long-term follow-up is mandatory even after radical resection, because recurrence or development of metastasis may be delayed many years.     

Giant cell tumor in queer location with lung metastasis!

Giant cell tumor (GCT) of bone accounts for ∼ 5 % of primary bone tumors, however involvement of rib is uncommon. We here discuss a rare case of giant cell tumor of anterior arc of rib mimicking malignant breast mass with associated lung metastasis in a 28 year old woman. CECT and MRI revealed large soft tissue mass with epicenter at 3rd rib and erosion of 3rd rib. CECT also revealed lung metastasis. Histopathology confirmed the diagnosis of giant cell tumor.     

A case of hemangiopericytoma in the pelvic retroperitoneum and review of literature of hemangiopericytoma in the retroperitoneal space in Japan

Hemangiopericytoma is a rare capillary tumor that was first described and named by Stout and Murray in 1942. A case of hemangiopericytoma in the pelvic retroperitoneum is reported. The patient, a 57 year old man, was admitted because of dysuria. He underwent extirpation of the tumor with the rectum by low anterior resection. The pathological diagnosis was malignant hemangiopericytoma, and Adriamycin was administered to prevent recurrence or metastasis. He has been free from any recurrence or metastasis for the last eight months. Twenty cases of hemangiopericytoma in the retroperitoneum including our case, were collected from Japanese literature and reviews. Hemangiopericytoma, especially in the retroperitoneal space, should be regarded as a highly malignant tumor. Early aggressive therapy and long term follow up are recommended because the tumor characteristically produces late local recurrence and metastasis.     

Giant retroperitoneal pelvic hemangiopericytoma.

Hemangiopericytoma is a rare soft tissue sarcoma, comprising less than one percent of all vascular neoplasms. This report describes a 75-year-old man with a very large malignant retroperitoneal hemangiopericytoma that was successfully excised. The tumor measured 23 cm in its greatest dimension and, as such, is the largest malignant hemangiopericytoma reported to date. The clinical and pathologic features of this lesion and a review of the literature are presented.     

Intracranial meningeal hemangiopericytoma metastatic to the scapula

Meningeal hemangiopericytomas are rare vascular tumors that have a propensity for recurrence and metastasis. Intracranial hemangiopericytomas are rare vascular tumors. They account for 0.5% of primary central nervous system tumors and 2% of meningiomas. Unlike usual benign meningiomas, which rarely metastasize extracranially, meningeal hemangiopericytoma has a high rate of local recurrence and distant metastasis. The treatment paradigms for hemangiopericytomas and meningiomas differ based on their biological behaviors. Hemangiopericytomas have higher rates of recurrence and metastasis compared with meningiomas. Intracranial meningeal hemangiopericytoma is characterized by clinically repeated local recurrences at the primary site. Bone, liver, lung, central nervous system, and abdominal cavity are the most commonly reported sites of metastasis in hemangiopericytomas.This article describes a case of bone metastasis with extensive involvement of the scapula from intracranial hemangiopericytoma. Bone metastasis can be seen in a relatively late phase of the disease, with metastasis to other organs. Although radiation therapy is effective in controlling pain from bone metastases in unresectable disease and those with extensive visceral metastases, aggressive local surgical control of a solitary bone metastasis may be an option for patients with limited distant disease. The diagnosis may be initially confused with clear cell meningioma and benign meningiomas. The management of bone metastasis is not well reported in the orthopedic literature.     

Metastatic osteosarcoma causing intussusception

Osteosarcoma is the most common malignant bone neoplasm. It has a very high metastatic potential, and lungs are the most common site of metastasis followed by bones. Multiagent chemotherapy has been reported to alter the biological behavior of the disease so that the tumor metastasizes to more rare sites such as brain, bowel, and soft tissues. The authors report a case of jejuno-jejunal intussusception caused by metastases from osteosarcoma.     

Giant fibrosarcoma arising in dermatofibrosarcoma protuberans on the scalp during pregnancy

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence; however, it seldom metastasizes. Most lesions occur over the trunk or proximal extremities, and there is slight male preponderance. DFSP is rare on the head and neck. Less than 5% tumors are located on the scalp. OBJECTIVE: To describe a case of giant fibrosarcoma arising in DFSP on the scalp during pregnancy. METHOD: In our patient, the tumor was on scalp and measured 15 x 11 x 7 cm. The recurrent tumor that developed in the same region was measured 33 x 30 x 15 cm. Histopathologic examination of this mass revealed fibrosarcoma. The mass was excised from the subperiosteal plane with a 3-cm free margin. Because of the high possibility of recurrence, the defect site was reconstructed with a split-thickness skin graft. RESULTS: After 1 year of follow-up, no recurrence or metastasis was detected. CONCLUSION: We present a unique case in which the person has a giant fibrosarcoma on her scalp arising from DFSP with fibrosarcomatous change during pregnancy.     

Ossifying fibromyxoid tumor of oral cavity

Ossifying fibromyxoid tumor is a rare tumor of mesenchymal origin with varied presentation at different sites including head and neck. Clinically these are slow growing lesions and patients have a variable age at presentation. A 14 years old girl presented with a slowly enlarging gingival swelling, which on radiological examination showed increased rarefaction in the mandible with a provisional diagnosis of an inflammatory lesion. Microscopically, the tumor had spindle to oval shaped cells in a fibromyxoid background with a peripheral shell of lamellar bone. Histological diagnosis of ossifying fibromyxoid tumor was made after immunohistochemical stains for vimentin and S-100 protein. Recurrence, metastasis and histologically increased mitotic count are indicative of atypical or malignant ossifying fibromyxoid tumors.     

The sarcomatous guise of cervical ectopic hamartomatous thymoma

BACKGROUND: Ectopic hamartomatous thymoma is a rare benign neoplasm occurring in the deep soft tissues adjacent to the sternoclavicular joint. Although clinical presentation and diagnostic imaging can be consistent with a malignant lesion such as a sarcoma, recognition of pathologic features can readily exclude such a diagnosis. However, this remains a challenge caused by their unusual histologic features and diverse composition. Recognition of this tumor is, however, important, because it follows a benign clinical course, and conservative surgical excision is the therapy of choice. METHODS: A literature review of all reports of ectopic hamartomatous thymoma was undertaken and compared with our indexed case. RESULTS: From this review, we identified 26 previous reports. Most patients were men, with a 4.5:1 predominance. There was a wide range of ages at presentation, from 26 to 79 years. All tumors were located in the lower neck, with the exception of one arising presternally. Treatment predominantly consisted of tumor resection, with no reported recurrences on follow-up. CONCLUSIONS: We conclude that ectopic hamartomatous thymoma is an extremely rare neoplasm usually presenting in the lower neck. Correct identification of this tumor is important, because it follows a benign course, and surgical excision is adequate therapy.     

Chondroblastoma of the femoral neck as a cause of hip synovitis

Chondroblastoma is a rare benign chondroid bone tumor, which typically arises from the epiphysis of a long tubular bone. We present a 13-year-old girl with persistent hip synovitis caused by a chondroblastoma that arose from the femoral neck (metaphysis) and uniquely extended extraosseously into the hip joint. Computed tomography scan clearly demonstrated a tumoral lesion of the left femoral neck and a periosteal reaction along the anterior cortex of the femur, distal to the legion. Magnetic resonance imaging showed reactive synovitis with marked joint effusion of the hip and an extensive edema in the proximal femur and the surrounding soft tissues. Aneurysmal bone cysts formation secondary to chondroblastoma was confirmed by histologic examinations of the excised specimens. Complete relief of pain and an improvement in the range of movement of the hip joint was obtained after surgical resection of the tumor.     

Merkel cell carcinoma with bone metastasis: a case report

 A case of Merkel cell carcinoma with bone metastasis is described. The patient, who had a history of Merkel cell carcinoma of the skin in the right cheek, had spontaneous pain in the right thigh. At the initial visit, the right hip range of motion was slightly limited, but there was no gait disturbance or abnormality in the radiographs of the right hip. However, the pain gradually increased and caused gait disturbance. The patient underwent surgical treatment. A bipolar type of femoral prosthesis was implanted into the femur, and sampling of cancellous bone was performed at the time of osteotomy. Pathological examination showed the findings of Merkel cell carcinoma. Merkel cell carcinoma is a rare malignant tumor of the skin, which usually occurs on the head, neck, or extremities and metastasizes to the lymph nodes. Although osseous involvement often occurs in the adjacent facial bones through direct invasion, distant osseous metastasis appears to be extremely rare. Received: November 12, 2001 / Accepted: April 18, 2002     

Secondary chondrosarcoma in cartilage bone tumors: report of 32 patients

Background Secondary malignancies arising from benign bone tumors are rare. Their recognition and diagnosis are difficult, and their slow growth and late recurrence require long-term follow-up. In this study, malignant transformation rates of various histological types of benign cartilage-forming bone tumors in large series were evaluated. Methods Between 1986 and 2004, a retrospective analysis of 627 cartilage-forming benign bone tumors revealed that 32 patients had malignant transformation. Of the 32 patients, 14 had solitary osteochondromas, 10 had multiple osteochondromas, 6 had a solitary enchondroma, 1 had Ollier's disease, and 1 had Maffucci's syndrome. The patient with Ollier's disease had two chondrosarcomas, and one patient with multiple osteochondroma had three chondrosarcomas. The cases were included in the study only when complete clinical documentation, radiological records, and histological analyses were available. Results The rate of malignant transformation for cartilage-originating tumors was 5.1% (solitary osteochondromas 4.2%, multiple osteochondromas 9.2%, solitary enchondromas 4.2%). The average time between the initial diagnosis and malignant transformation was 9.8 years. The most common site of involvement was the proximal portion of the femur. The tumors generally were well differentiated. The mean follow-up period was 57.3 months. Five patients (15.6%) died of tumor recurrence or metastasis at an average of 20.6 months. One patient is alive with tumor at 104 months. Conclusions Cartilage-forming benign bone tumors are rather prone to undergo malignant transformation. Although malignant transformation of a benign bone tumor is a rarely encountered situation, orthopedic surgeons should be cautious while following patients with a benign bone neoplasm. Early recognition and appropriate surgical treatment are required to achieve successful outcomes. The rate of local recurrence in secondary chondrosarcomas depends not only on adequate surgical treatment but also on the localization and histological grade.     

Prostate cancer metastasized to thyroid cartilage: a case report

A 75-year-old male was diagnosed as prostate cancer (serum PSA: 4,772 ng/ml, Gleason score: 4 + 4 = 8) with multiple bone metastases. And he noticed a painless mass of the frontal neck a month before the diagnosis. Computed tomography of the neck showed a tumor in the thyroid cartilage. Biopsy of the neck tumor revealed metastasis of prostate cancer by positive PSA staining. Metastasis of malignant tumor to cartilaginous tissue is extremely rare because there are usually no vessels in it. Only 4 cases of the metastasis of prostate cancer to the thyroid cartilage have been reported. It was thought that tiny bone marrows were formed in the ossified cartilage and it caused hematogenous metastasis.     

Desmoid tumor: a case of mistaken identity

Desmoid tumors are rare tumors accounting for only 0.03% of all neoplasms. Mainly occurring in the fourth and fifth decades of life, these tumors originate in musculoaponeurotic tissues of the limbs, neck, trunk, abdominal wall, and mesentery. We present a rare case of a chest wall desmoid tumor that was mistaken for breast cancer on both physical examination and mammography, which highlights the unique risk these tumors present for confusion with other malignant processes. Although past literature contains numerous reports of other misdiagnoses, this case is unique in reporting the potential for misdiagnosis between chest wall desmoid tumors and breast cancer. In cases where suspicious breast findings do not correlate to usual diagnostic measures, such as fine-needle aspiration or core needle biopsy, the possibility of another pathology such as a chest wall desmoid tumor mimicking breast cancer should be considered in the differential diagnosis.     

Distinctive Head and Neck Bone and Soft Tissue Neoplasms

Benign and malignant primary bone and soft tissue lesions of the head and neck are rare. The uncommon nature of these tumors, combined with the complex anatomy of the head and neck, pose diagnostic challenges to pathologists. This article describes the pertinent clinical, radiographic, and pathologic features of selected bone and soft tissue tumors involving the head and neck region, including angiofibroma, glomangiopericytoma, rhabdomyosarcoma, biphenotypic sinonasal sarcoma, chordoma, chondrosarcoma, and osteosarcoma. Emphasis is placed on key diagnostic pitfalls, differential diagnosis, and the importance of correlating clinical and radiographic information, particularly for tumors involving bone.     

Soft Tissue Tumors Rarely Presenting Primary in Bone; Diagnostic Pitfalls

Primary bone sarcomas represent extremely rare entities. The use of now abolished labels, such as malignant fibrous histiocytoma and hemangiopericytoma, has significantly hampered the chance of identifying specific entities. It is now accepted that a broad variety of mesenchymal malignancies most often arising on the soft tissue may actually present as primary bone lesions. A more accurate morphologic partition is justified based on availability of distinct therapeutic options. An integrated diagnostic approach represents the only way to achieve a correct classification. In consideration of the significant complexity, primary bone sarcomas should ideally be handled in the context of expert centers.     

Primary alveolar soft-part sarcoma of the liver: anomalous presentation of a rare disease

Alveolar soft-part sarcoma is a highly vascular soft-tissue tumor that is uniformly malignant. It comprises less than 1 per cent of all soft-tissue sarcomas. Patients with alveolar soft-part sarcoma most frequently are aged 15 to 35 years, and the soft tissues of the lower extremities typically are affected. In the pediatric population, it most frequently occurs in the head and neck and particularly affects the tongue and orbit. Alveolar soft-part sarcoma has been described as a primary lesion in the trunk, upper extremities, and retroperitoneum; more novel locations include the mediastinum, female genital tract, stomach, bone, and larynx. Numerous case reports describe alveolar soft-part sarcoma in diverse anatomic locations, but this report is, to our knowledge, the first documentation of primary alveolar soft-part sarcoma of the liver. We describe a 47-year-old woman with such a manifestation. Despite surgical resection and numerous chemotherapeutic regimens, this patient had widespread metastasis and died approximately 2 years after the diagnosis was established.     

人工间置型假体置换治疗长骨骨干恶性肿瘤（附4例报告）

目的探讨人工间置型假体置换治疗长骨骨干恶性肿瘤的手术方法与疗效。方法 2008年6月-2010年6月,4例长骨骨干恶性肿瘤患者于我院接受骨干瘤段切除人工间置型假体置换手术。4例均为男性,年龄24～60岁,平均41岁。其中股骨干恶性纤维组织细胞瘤1例,肺癌单发股骨干转移瘤1例,软组织腺泡状肉瘤胫骨干转移瘤1例,肾癌单发肱骨干转移瘤1例。结果获得随访16～40个月。截止末次随访时,4例患者患肢功能良好,MSTS评分平均为28.3分（27～30分）。均无局部复发。其中3例无瘤生存;1例胫骨干转移瘤患者术前已有肺转移,术后9个月出现脑转移,目前带瘤存活。结论肿瘤瘤段截除人工间置型假体置换术是治疗长骨骨干恶性肿瘤的有效术式之一,该术式能够达到安全的外科边界,降低局部复发率,并保留良好的肢体功能。     

Foot malignancies: a multidisciplinary approach

Malignant osseous and soft tissue tumors of the foot are rare and should be considered in patients who have foot-related symptoms. Most sarcomas affect patients who are older than 55 years; however, many young patients who are otherwise healthy, may present with malignant foot and ankle lesions. In addition to benign and malignant neoplasms, pseudotumorous conditions mimic neoplastic lesions and should be differentiated before any treatment is undertaken. Invasiveness is a characteristic of sarcomas. The foot is a terminal anatomic structure, with closed and tight compartments and well-vascularized tissues, that predispose it to hematogenous and lymphatic metastases. Patients who have a malignant foot tumor should be referred to an orthopedic oncologist for further evaluation and treatment. The goals of treatment include local tumor control, restoration of function and stability during standing and walking, long-term survival, and improved quality of life. In most cases, wide surgical margins require a ray, Syme, midtarsal, or below-the-knee amputation. Recent advances in chemotherapy and radiotherapy have allowed limb salvage procedures with wide tumor resections. Because overall survival is greatly improved in these patients, the reconstruction of skeletal defects needs to be more functional and durable. After tumor resection, reconstruction of the skeletal and soft tissue defects is possible by using bone allografts or vascularized autografts, arthrodesis, and free vascularized musculocutaneous flaps.     

Paraganglioma of the jugulo-tympanic glomus. Anatomo-pathological aspects

15 extensive glomus jugulare tumors have been studied. Optic and ultrastructural data were very similar to those which have been reported in the literature. They possessed a local aggressiveness, demonstrated by progressive spreading in soft tissues and bone, intravascular digitations and destruction of cranial or sympathetic nerves. Dura-mater was usually preserved and prevented extension close to the brain stem. In this group, no functional activity of catecholamine-secreting tumor nor metastasis proving a malignant form were observed.