Manabu Hoshi

Malignant change secondary to fibrous dysplasia.

BackgroundMalignant change in fibrous dysplasia (FD) is very rare. This study was carried out to establish some characteristic clinical information about this disorder.MethodsFour cases with a malignant change in FD out of 128 cases with FD were surgically treated and followed up for a median period of 61.3 months. The mean age of the patients was 39.8 years. Clinical features, radiological findings, and the outcome were analyzed for each of the four cases.Results and conclusionThe sites of the lesions were tibia (2 cases), femur (1 case), and rib (1 case). The forms of FD were monostotic in one case and polyostotic in three cases. Radiologically, plain films and computed tomography (CT) showed osteolytic lesions with poorly delineated margins within and/or near areas having a ground-glass appearance. In the osteolytic lesions, simple cystic changes associated with old FD could be excluded by enhanced magnetic resonance imaging (MRI). Histopathologically, two cases were osteosarcoma, one case was malignant fibrous histiocytoma (MFH), and one case was fibrosarcoma. The management of this disease should be decided according to the type of primary high-grade bone sarcoma. One patient, with MFH, was dead of lung metastasis 13 months after surgery. The others are alive without disease.

A case of multiple skeletal lesions of brown tumors, mimicking carcinoma metastases

Brown tumor is not a true tumor, being an unusual reactive lesion in association with primary or secondary hyperparathyroidism. We report a 23-year-old woman, who initially presented with lower back pain caused by ureterolithiasis. The initial diagnosis of brown tumor was delayed, but later pain in her leg worsened and a sacral lesion was incidentally discovered on lumbar magnetic resonance imaging (MRI); multiple destructive bone lesions were then found radiologically. The radiological features of the multiple bone lesions, which mimicked multiple metastatic tumors, seemed to be those of the terminal stage of malignancy. However, pathological examination and abnormal laboratory data showing elevated serum calcium, alkaline phosphatase, and parathyroid hormone and low serum phosphate confirmed the diagnosis of brown tumor. Adenoma in the parathyroid gland was confirmed and surgically resected. The clinical symptoms of bone pain, and abnormal radiological findings and laboratory data were resolved 6 months after surgery. Synthetic analysis of the clinical, radiological, and laboratory findings was necessary for the definite diagnosis of brown tumor.

Clinical Problems After Initial Unplanned Resection of Sarcoma

OBJECTIVE Unplanned resection of a sarcoma is often chosen in the early phase by general physicians without any imaging scrutiny. The present study aimed to highlight the clinical problems associated with unplanned resection of sarcomas. METHODS Thirty-eight patients who underwent unplanned resection of a sarcoma and additional treatment were examined. The definite histological grading was high in 31 patients and low in 7 patients. RESULTS The tumors were located in the depth of the subfascia in 13 patients. The maximal tumor sizes exceeded 5 cm in 16 patients. Preoperative MRI was only performed in six patients. The previous surgical margins were intralesional in 20 patients and marginal in 18 patients. Inappropriate transverse skin incisions were found in 21 patients. Extensive hematoma at the initial surgical site was seen in five patients. Thirty-three patients accepted additional wide resection due to the insufficient removal of malignancy. During an average follow-up of 42.7 months, seven patients died of lung and brain metastases. CONCLUSIONS On excision of any soft tissue tumor, surgeons should be aware of the potential risk for erroneous management of malignancy. If not, careless surgery may render the treatment protocol complicated and require excessive additional tissue resection with poor function and prognosis. Appropriate salvage treatment may have a significant role to play after unplanned resection of the sarcoma.

A Japanese patient with Li-Fraumeni syndrome who had nine primary malignancies associated with a germline mutation of the p53 tumor-suppressor gene

We describe a patient who had nine primary malignant tumors and a germline mutation in the p53 tumor-suppressor gene, characteristically found in the Li-Fraumeni syndrome (LFS). A 15-year-old girl with no family history of cancer was referred to our hospital because of pain and swelling of the right knee. Osteosarcoma was diagnosed. The patient received chemotherapy followed by surgery and had a remission. After the age of 28 years, nine primary malignant tumors developed successively, including right breast cancer, colon cancer, malignant fibrous histiocytoma (MFH) of the abdominal wall, right lung double cancers, bilateral breast cancers, and MFH of the left thigh. This is the second highest number of types of primary malignant tumors to be reported in LFS. All tumors were treated by a multidisciplinary approach, including surgery. Genetic analysis revealed a germline missense mutation in the p53 gene (c.659 A > G), resulting in Y220C, which has been reported in three families with LFS. The patient died of lung metastasis from MFH at the age of 37 years. Despite the multiple tumors, repeated induction of remissions resulted in long survival. Our findings suggest that a multidisciplinary approach to treatment, including surgery, is beneficial in patients with LFS.

Oncologic outcome of parosteal osteosarcoma.

BackgroundThis retrospective review evaluated the clinical features and surgical outcomes of parosteal osteosarcoma (POS).MethodsNine patients were surgically treated and followed up. Their mean age was 30.8 years. Clinical information and oncologic outcomes of each case were analyzed.ResultsSites of involvement were all in the femur, and all tumors arose from the metaphyseal area of the distal femur. Biopsies for definite diagnoses were performed in just two of the nine cases. Wide resection was applied for all tumors. Surgical evaluations were a 1-cm-wide procedure in six cases and a 2-cm-wide procedure (or more) in three cases. All patients were found to be continuously disease free during the follow-up period of 115.1 months.ConclusionPOS showed characteristic findings on radiographic images. Therefore, wide resection without biopsy could be performed in 77.8% (7/9) of the cases. This procedure may contribute to attaining better limb function, because of preventing contamination of healthy surrounding tissue and minimizing the extent of resection. The safety margin was evaluated as a 1-cm-wide procedure. For the choice of reconstruction, indication of autobone grafting (3/9) or total knee replacement (TKR) (6/9) depended on tumor size, location, and shape. With no adjuvant treatments, all cases have shown good clinical courses during the entire follow-up period of about 10 years.

Role of FDG-PET/CT for monitoring soft tissue tumors

The aim of the current study was to evaluate the limitations of 2-deoxy-2-F18-fluoro-D-glucose positron emission tomography combined with computed tomography (FDG-PET/CT) when monitoring soft tissue tumors. The diagnostic criteria of malignancy was defined as the tumor having a maximum standardized uptake value (SUVmax) ≥2.0 and a maximum diameter ≥5 cm as measured using FDG-PET/CT. One-hundred-and-thirteen patients, that were either included in the criteria or not, were compared. In addition, the values of SUVmax of the primary tumor and relapse in 12 patients were evaluated. The Kaplan-Meier analysis demonstrated that patients with tumors measuring ≥5 cm size and ≥2.0 SUVmax were associated with a worse survival rate. Among the 12 patients with relapse, statistical significances were detected in the tumor diameters, however, not in the SUVmax values. Thus, the criteria identified patients that were associated with a poor prognosis, and the SUVmax of distant metastases and local recurrences were identified to be significantly affected by tumor size.

A Comparative Study of Clinicopathological Features Between Simple Bone Cysts of the Calcaneus and the Long Bone

Background: The potential unrevealed clinicopathological differences between simple bone cysts situated in the calcaneus (calcaneal bone cysts) and those situated in long bones (long bone cysts) were investigated in the present study. Methods: A total of 41 cysts from 41 patients who underwent operative treatment were evaluated: 16 cysts in the calcaneus, 17 in the humerus, 7 in the femur, and 1 in the tibia. The clinical course, radiological findings, and histological features were retrospectively reviewed. Results: The mean patient age was 11.5 years (range, 3 to 25 years), and the mean follow-up was 33.0 months (range, 12 to 77 months). The mean patient age was significantly higher in patients with calcaneal bone cysts (13.5 years; long bone cysts, 10.2 years, P < .05). No treatment failure was seen for patients with calcaneal bone cysts, while 7 long bone cysts required reoperation. In H&E-stained specimens of the cyst wall, cholesterol clefts were identified in 10 of the 16 calcaneal bone cysts (62.5%), whereas none were identified in long bone cysts (0%; P < .0001). Conclusion: Our study elucidates the different clinicopathological features existing between calcaneal bone cysts and long bone cysts. Cholesterol clefting is most likely due to hemorrhaging and the subsequent breakdown of blood in the cyst. The significance of these differences between long bone and calcaneal cysts is still open to conjecture. Level of Evidence: Level III, retrospective comparative series.

Intraosseous schwannoma of the proximal femur

A schwannoma is a benign nerve sheath tumor commonly located in soft tissue. An intraosseous schwannoma is very rare, and only four cases involving the femur were found in a review of the English‐language medical literature. We present a rare case of a schwannoma with well‐defined, lytic, dumbbell‐shaped and benign‐appearing lesions of the proximal femur.

Bone metastasis of intracranial meningeal hemangiopericytoma

The authors investigated the clinical and radiological features and the treatment strategy for bone metastasis from a rare tumor, intracranial meningeal hemangiopericytoma. The clinical presentations and characteristic imagings were retrospectively reviewed in 15 bony metastatic lesions of four patients with this tumor. All four cases were initially diagnosed as atypical meningioma, and all of the bone metastases developed more than 10 years after the initial intracranial surgery. The common symptom induced by the metastatic lesions was pain. On plain roentgen films and computed tomography (CT), the involved bones showed thinning and expansion. On bone scintigraphy, a “cold-in-hot” appearance was typically observed. The clinical and radiological findings were diagnosed as bone metastasis from intracranial meningeal hemangiopericytoma. The prognosis after bone metastasis was relatively short compared to the long duration before bone metastases, because of the coexistence of other, visceral, metastasis. Combined with effective radiation therapy, surgical intervention for bone metastasis of this tumor should be carefully considered.

A case of dedifferentiated liposarcoma showing a biphasic pattern on 2-deoxy-2-F18- fluoro-D-glucose positron emission tomography/ computed tomography

Abstract Integrated 2-deoxy-2-F18-fluoro-D-glucose positron emission tomography combined with computed tomography (FDG-PET/CT) has been used in the field of soft tissue sarcoma. We report an 81-year-old man with dedifferentiated liposarcoma in the left thigh, which was composed of well-differentiated liposarcoma and pleomorphic malignant fibrous histiocytoma. As well as other radiological modalities, FDG-PET was able to demonstrate a biphasic signal pattern composed of well-differentiated liposarcoma and dedifferentiated area, being consistent with the histological grade of malignancy.