双处截骨纵向搬移治疗长段小腿感染性复合缺损

目的探讨胫骨双处截骨纵向搬移治疗胫骨干大段感染性骨缺损复合软组织缺损的疗效及并发症情况。方法回顾性分析2009年5月至2016年5月收治的31例胫骨干大段感染性骨缺损复合软组织缺损资料,男27例,女4例,年龄2~58岁,平均33.4岁。胫前软组织缺损面积7 cm×8 cm~24 cm×12 cm,胫骨缺损长度6.5 cm~18.2 cm,平均11.4 cm。术后随访时间1~8年,平均32个月。随访观察骨及软组织愈合和并发症情况。结果31例感染均愈合,骨缺损及软组织缺损创面全部愈合。4例出现骨断端延迟愈合,植骨后愈合,3例出现骨延长区伤口感染及成骨不良,经放置万古霉素骨水泥棒2个月及"手风琴"技术处理后愈合;其余骨缺损与软组织创面均顺利愈合。软组织愈合时间为65 d~190 d,平均70 d,骨愈合的时间为14~35个月,平均21.6个月;28例患者肢体长度完全恢复,3例患者患肢较健侧短1.5 cm。结论对于8 cm以上的胫骨骨与软组织复合缺损,双处截骨纵向搬移可缩短治疗时间。主要并发症为骨会师端延迟愈合、成骨不良和胫骨力线偏移,经处理后恢复正常。     

Phosphaturic mesenchymal tumor (mixed connective tissue variant): a case report with spectral analysis

We present a further case of a rare mesenchymal neoplasm termed phosphaturic mesenchymal tumor (mixed connective tissue variant). The patient was a 42-year-old man with a long history of osteomalacia of unknown etiology with pathological bone fracture, abnormality of parathyroid glands, kyphosis, scoliosis, and spondylosis. Laboratory investigation disclosed hypophosphatemia, elevated serum alkaline phosphatase activity, and normal serum calcium level. The patient had a soft tissue mass in the right inguinal area, measuring 11×6×5 cm, which was previously interpreted as a calcified hematoma on sonography. The tumor was surgically removed. Grossly, the tumor was well circumscribed, unencapsulated, and had soft to dense consistency. The cut surface had a variegated appearance due to the presence of large hemorrhagic areas admixed with foci of grey-yellow tissue. Histologically, the tumor was composed of primitive mesenchymal cells, osteoclast-like cells, and cells showing myofibroblastic features without cytologic atypia. There were a well developed vascular network, microcystic areas, and poorly formed cartilaginous foci. Unusual and hitherto unpublished prominent features were flower-like, slate-gray crystals, widespread hemosiderin deposits and large areas of hemorrhages, with the latter comprising approximately 60% of the tumor. A spectral analysis indicated that chemically, the crystals mainly consisted of calcium phosphate and sodium nitrate.     

Sclerosing hemangioma with florid endobronchial and endobronchiolar growth

Sclerosing hemangioma (SH) with endobronchial growth (SH-EG) is an extremely unusual form of SH. A case of SH-EG in a 47-year-old female is described. She suffered from a productive cough for 4 months. A chest CT scan revealed a well-circumscribed, parenchymal mass with endobronchial lesions continuously extending to the right main bronchus. Right upper sleeve lobectomy was carried out. The tumor was 4.8 × 3.5 cm in size, tan-brown, and elastically soft. It was located in the pulmonary parenchyma of the right upper lobe and continuously extended to the right upper bronchus. Furthermore, the main tumor also spread into segmental bronchi and peripheral bronchioles. Microscopically, the tumor consisted of round to cuboidal cells with papillary and solid patterns, partly showing sclerosis and hemorrhage. For 2.5 years after surgery, the patient has been well without recurrence or metastasis. Florid intrabronchial extension as seen in this case has never been documented in SH. To form an endobronchial component, it seems to be crucial that the parenchymal SH is located adjacent to the bronchus and involves it, followed by the destruction of the bronchial cartilage.     

PET/CT对小动物前列腺癌骨转移的观察研究

目的　探讨小动物PET/CT扫描在小鼠前列腺癌骨转移检测中的可行性。 方法　裸鼠23只,随机分为对照组2只,前列腺原位注射组、左心室注射组、胫骨腔内注射组每组各7只。分别采用前列腺原位注射、左心室内注射、胫骨腔内注射的方法建立前列腺癌骨转移动物模型。建模成功后饲养40天,原位注射及左心室注射组采用PET/CT扫描检测,胫骨腔注射组采用小动物高分辨率CT检测,对可疑的骨转移灶行解剖学观察及HE染色明确。 结果　前列腺原位注射组肿瘤细胞聚集腹腔软组织内生长,均未发现明显骨质破坏（0/7）;左心室注射组均发生皮下等软组织转移（7/7）, PET/CT检测出一例胫骨上端骨质破坏,组织学检测证实为骨转移灶(1/7);胫骨腔注射组所有动物均形成明显骨质破坏（7/7）且高分辨率CT检测见骨破坏分级良好。 结论　小动物PET/CT扫描能够良好的显示转移灶在小鼠体内的定位,且能良好的显示骨破坏情况,因此该技术在检测小动物骨转移上有着良好的可行性。     

Case 9

A 66-year-old woman was shown on routine X-ray to have a well-circumscribed anterior mediastinal mass (Fig. 1). The mass was totally excised, and no extension was noted beyond the mediastinum. Grossly, the tumor measured 7.5 × 7.5 × 7.5 cm, weighed 102g, had a firm, gray cut surface, and appeared encapsulated. No further therapy was given. Recurrent disease was noted radiographically in the anterior mediastinum 5 years later. A sternal split revealed three tumor masses situated on the aortic arch, superior vena cava, and right pulmonary hi/us, respectively; the largest measured 10 × 10 × 5 cm, weighed 80 g, and, on section, was brown and firm, with hemorrhagic areas. Tumor was also noted on the visceral pleura of the right lung. A formal thoracotomy, performed 2 months later, identified a 4 × 3 cm anterior mediastinal mass attached to the right middle lobe of the lung and invading the pericardium. Many implants were noted on the posterior and lateral parts of the chest wall.

Throughout the course up to this point, the patient had been remarkably asymptomatic; there had been no symptoms or signs suggestive of any activity by the tumor. Three and one-half years following the last thoracotomy, massive local recurrence developed in the mediastinum and pleural spaces, which led to the death of the patient by respiratory insufficiency. There was no clinical evidence of distant metastases. Autopsy was not obtained.     

Plexiform perineurioma of the lip: A case report and review of literature

We describe a plexiform perineurioma, which is an extremely rare variant of soft tissue perineurioma. A 43‐year‐old Japanese man presented with a painless, well‐demarcated, elastic, soft tumor measuring 2.0 × 1.9 cm on the upper lip that had persisted for three years. Microscopically, a plexiform tumor composed of minimally atypical spindle cells with wavy nuclei was located in the lamina propria of the oral mucosa. Tumor cells were concentrically arranged around small vessels and aligned in parallel with delicate collagen fibers on a fibromyxoid background in the hypocellular area. Tumor cells were immunohistochemically positive for EMA, GLUT‐1, claudin‐1, and CD34 and negative for S‐100 protein. The histopathological and immunohistochemical profiles of the excised tumor indicated a diagnosis of plexiform perineurioma. The patient has remained free of recurrence for sixteen months. Intraoral soft tissue perineurioma is rare and two of the four reported plexiform perineuriomas, including that described herein, notably involved the intraoral area. According to previous reports, plexiform perineuriomas largely develop in middle‐aged women without a history of type 1 or type 2 neurofibromatosis. The clinical courses of all reported plexiform perineuriomas have been benign.     

Corpora amylacea in a leiomyoma of soft tissue

A case of myxoid leiomyoma of deep soft tissue is described. The patient was a 42-year-old woman who presented with menorrhagia, and an ischiorectal mass was identified. A total hysterectomy was performed, and the mass was removed. On gross examination, the mass was 6cm x 5cm x 4cm and had a mucoid cut surface. Histologic examination revealed a myxoid leiomyoma with abundant intracellular corpora amylacea (CA). An infarcted leiomyoma was present in the hysterectomy specimen. The patient remains well, with no evidence of recurrence 3 years after surgery. Leiomyomas of deep soft tissue are rare, and, to our knowledge, none have been described containing CA.     

Primary leiomyosarcoma of the submandibular gland: a case report

Background

Leiomyosarcoma is a rare malignant mesenchymal tumor that represents 5–7 % of all soft tissue sarcomas. The occurrence of this tumor in the salivary glands is exceptional. Only five cases are reported in the submandibular gland.

Case presentation

A 65-year-old white Moroccan woman presented with a history of progressive right submandibular swelling which had grown over a period of 8 months. Clinical examination showed a submandibular painless, mobile and hard mass without cervical lymphadenopathy. Ultrasonography and computed tomography revealed a solid and heterogeneous mass measuring 4?×?2 cm involving the submandibular gland. A resection of the gland was performed. Pathological findings were consistent with primary leiomyosarcoma of the submandibular gland. No recurrence occurred after two months of follow-up.

Conclusion

Primary leiomyosarcoma of the submandibular gland is an extremely rare mesenchymal tumor. Clinical and radiological features are not specific. Differential diagnosis includes metastatic leiomyosarcoma and gastrointestinal stromal tumor, myoepithelioma, sarcomatoid carcinoma, melanoma and other sarcomas. Little is known about the overall biologic behavior of this tumor, but the prognosis seems to be poor.

     

Atypische lipomatöse Tumoren des Magens

Two cases of primary gastric atypical lipomatous tumors (ALT) are presented. In case No.1, a 7×4×3 cm submucosal ALT (lipoma-like subtype) of the antrum/pyloric region in a 60-year-old woman was completely resected. Using interphase dual-color-FISH, MDM2- and CDK4 amplifications could be detected in distinguished amplicons. The patient was continuously free of disease after 56 months. In case No. 2, a 3.5 cm (in diameter) submucosal ALT (lipoma-like subtype) of the gastric body in a 56-year-old woman was completely resected. FISH revealed MDM2 amplification while the CDK4 gene remained in diploid copies. This patient was continuously free of disease after 36 months. The morphologic and molecular biological findings of this rare primary gastric mesenchymal tumor are discussed in comparison with the corresponding soft tissue lesions.     

股部鲍温病1例

报告1例股部鲍温病.患者男,80岁.右股部出现红色斑片10年就诊.皮肤科检查:右股部可见境界清楚的红斑,表面灰色结痂,湿润的糜烂面,面积约5 cm×8 cm大小.组织病理检查结果符合鲍温病.     

Investigating the Longitudinal Effect of Ovariectomy on Bone Properties Using a Novel Spatiotemporal Approach

Osteoporosis is the most common bone disease. However, the mechanism of osteoporosis-induced alterations in bone is still unclear. The aim of this study was to investigate the effects of osteoporosis on the structural, densitometric and mechanical properties of the whole tibia using in vivo μCT imaging, spatiotemporal analysis and finite element modeling. Twelve C57Bl/6 female mice were adopted. At 14 weeks of age, half of the mice were ovariectomized (OVX), and the other half were SHAM-operated. The whole right tibia was scanned using an in vivo μCT imaging system at 14, 16, 17, 18, 19, 20, 21 and 22 weeks. The image datasets were registered in order to precisely quantify the bone properties. The results showed that OVX led to a significant increase in the endosteal area across the whole tibia 4 weeks after OVX intervention but did not have a significant influence on the periosteal area. Additionally, the bone volume and mineral content significantly decreased only in the proximal regions, but these decreases did not have a significant influence on the stiffness and failure load of the tibia. This study demonstrated the application of a novel spatiotemporal approach in the comprehensive analysis of bone adaptations in the spatiotemporal space.     

Periosteal osteosarcoma of the femur with bone marrow involvement: A case report

Periosteal osteosarcoma is an exceedingly rare type of chondroblastic osteosarcoma, showing rather better prognosis, and secondary bone marrow involvement is unusual. A case of a 22 year old male with periosteal osteosarcoma of the right femur with an associated bone marrow lesion is presented. The juxtacortical tumor, 16 ×11 × 9 cm, was located on the bone cortex of the upper diaphysis and extended into the surrounding soft tissues. A minimal bone marrow lesion was present, although the bone cortex was quite intact. Microscopically, the tumor consisted exclusively of atypical chondroblastic cells with a small osteoblastic area. The bone marrow lesion, interestingly, contained both multiple nodules of well-differentiated chondrosarcomatous components and a few demarcated foci of atypical spindle cells producing a fine osteoid matrix. It was reasonable to conclude, therefore, that this tumor was a periosteal osteosarcoma with an unusual secondary bone marrow lesion rather than a conventional (central) chondroblastic osteosarcoma with soft tissue invasion. The patients good prognosis with no tumor recurrence or metastasis during more than 7 years follow-up after surgery supports this conclusion.     

病灶刮除术联合术后地舒单抗治疗股骨头颈部骨巨细胞瘤的临床疗效观察

目的 探讨病灶刮除术联合术后地舒单抗治疗股骨头颈部骨巨细胞瘤（GCT）的临床疗效。方法 回顾性研究。纳入2013年4月—2019年7月新疆医科大学第一附属医院骨肿瘤外科收治的6例股骨头颈部GCT患者的临床资料,其中男3例、女3例,年龄22~40（28±6）岁。Campanacci分级、影像学分级均为Ⅱ级。依据骨肿瘤国际保肢协会股骨近端GCT分区方法：H1区1例、H1+H2区5例。6例患者均采用改良Watson-Jones入路行病灶刮除植骨内固定手术,术后均接受规律性地舒单抗注射治疗。观察指标：观察手术时间、术中出血量,术后感染、股骨头坏死等短期并发症发生情况,术后辅助行走时间;监测地舒单抗治疗过程中有无不良反应;定期影像学检查,观察患者术后植骨成活情况,骨质破坏、吸收情况,瘤腔边缘成骨情况,内固定移位、断裂情况,以及GCT复发情况;术后6个月采用国际骨肿瘤协会（MSTS）骨与软组织肿瘤术后功能重建评估标准评定患者下肢功能。结果 患者均顺利完成手术,手术时间150~215 min,平均179 min;术中出血193~1 400 mL,平均687 mL。术后伤口均一期愈合,未出现感染、骨折、股骨头坏死等短期并发症。6例患者均获得随访,随访20~96个月,平均48个月。患者术后4~6周可拄双拐部分负重活动,8~12周可拄单拐负重,术后3~13个月弃拐完全负重。6例患者地舒单抗皮下注射次数6~18次,平均12次;停药后继续随访6~81个月,平均37个月。随访期间定期复查X线片,瘤壁骨化满意时间6~15个月,平均11个月;实验室检查未见血钙、血磷指标异常,无用药相关不良事件。随访期间患者无骨质破坏、感染、骨折及内固定移位、断裂等相关并发症方式,未见GCT复发。术后6个月MSTS评分26~30分,平均27分,6例患者疗效评定均为优。结论 病灶刮除术联合术后地舒单抗治疗股骨头颈部GCT,可促进术后瘤腔边缘骨化,疗效满意。     

Central low-grade osteosarcoma with pagetoid bone formation: a potential diagnostic pitfall.

Central low-grade osteosarcoma is an uncommon form of osteosarcoma, which is often difficult to distinguish from benign bone lesions. We reviewed the radiographic studies, the histologic material and the clinical records of two patients with central low-grade osteosarcoma that closely simulated the histologic appearance of Paget's disease of bone. The patients were two women aged 46 and 53 years. Radiologically, they presented a large ill-defined densely sclerotic lesion involving the proximal tibia. Both lesions only focally presented the conventional histologic appearance of central low-grade osteosarcoma, with a proliferation of fibroblast-like cells embedded in a dense collagenous stroma and irregular anastomosing tumor bone trabeculae. The most striking feature was the presence of extremely thickened irregular plates of bone with an irregular mosaic pattern of cement lines that closely resembled that of Paget's disease of bone. One patient, who had been initially treated for Paget's disease for 7 years, experienced disease progression. At resection of proximal tibia, there was evidence of dedifferentiation to high-grade osteosarcoma. After 2 months, she developed local recurrence that was treated with above-knee amputation, followed by chemotherapy. She died with multiple lung metastases 4 months later. The other patient is alive 9 months after wide tumor resection. These cases further expand the spectrum of central low-grade osteosarcoma, and underscore the diagnostic difficulties in separating central low-grade osteosarcoma from benign bone diseases, which may lead to delay in diagnosis, inadequate treatment, and eventually to dedifferentiation. Recognition of this variant of central low-grade osteosarcoma is based on the aggressive radiologic appearance and on adequate tumor sampling for histologic examination.     

Effects of letrozole on breast cancer micro-metastatic tumor growth in bone and lung in mice inoculated with murine 4T1 cells

Breast cancer (BC) is the leading cancer in women worldwide. Metastasis occurs in stage IV BC with bone and lung being common metastatic sites. Here we evaluate the effects of the aromatase inhibitor letrozole on BC micro-metastatic tumor growth in bone and lung metastasis in intact and ovariectomized (OVX) mice with murine estrogen receptor negative (ER?) BC cells inoculated in tibia. Forty-eight BALB/c mice were randomly assigned to one of four groups: OVX, OVX + Letrozole, Intact, and Intact + Letrozole, and injected with 4T1 cells intra-tibially. Letrozole was subcutaneously injected daily for 23 days at a dose of 1.75 µg/g body weight. Tumor progression was monitored by bioluminescence imaging (BLI). Following necropsy, inoculated tibiae were scanned via µCT and bone response to tumor was scored from 0 (no ectopic mineralization/osteolysis) to 5 (extensive ectopic mineralization/osteolysis). OVX mice had higher tibial pathology scores indicative of more extensive bone destruction than intact mice, irrespective of letrozole treatment. Letrozole decreased serum estradiol levels and reduced lung surface tumor numbers in intact animals. Furthermore, mice receiving letrozole had significantly fewer tumor colonies and fewer proliferative cells in the lung than OVX and intact controls based on H&E and Ki-67 staining, respectively. In conclusion, BC-inoculated OVX animals had higher tibia pathology scores than BC-inoculated intact animals and letrozole reduced BC metastases to lungs. These findings suggest that, by lowering systemic estrogen level and/or by interacting with the host organ, the aromatase inhibitor letrozole has the potential to reduce ER? BC metastasis to lung.     

Melanotic medullary thyroid carcinoma: A case report with review of the literature

Melanotic medullary thyroid carcinoma is morphologically defined by the presence of melanin deposits in the cytoplasm of tumor cells. It is an extremely rare variant with only 15 cases described in the literature to date and only one report of diagnosis by fine needle aspiration (FNA) biopsy. A 51-year-old woman presented with neck swelling. An ultrasound examination revealed a single solid nodule in the right thyroid lobe that measured 5.4 × 4.7 × 4.3 cm. Laboratory examination revealed elevated levels of serum calcitonin (8643.0 pg/ml), carcinoembryonic antigen (CEA) (86.2 ng/ml), and chromogranin A (123.2 ng/ml). An FNA biopsy of the thyroid nodule revealed predominantly single plasmacytoid cells with round to oval eccentric nuclei and dark brown intracytoplasmic granules. Immunohistochemical studies with Melan-A performed on a cell block slide confirmed that the granules contained melanin. The tumor cells were also positive for calcitonin, CEA, synaptophysin, AE1/AE3, CAM5.2, and HMB-45(focal); the tumor cells were negative for chromogranin, thyroglobulin, PAX8 and TTF-1. The diagnosis was reported as melanotic variant of medullary thyroid carcinoma. The patient underwent a total thyroidectomy which revealed tumor cell expression of insulinoma-associated protein 1 and confirmed neuroendocrine differentiation. Shortly after she presented with tumor recurrence in the thyroidectomy bed. The tumor cells were positive for only S100, SOX10, and Melan-A. Molecular analysis with the SEMA4 Solid Tumor Panel revealed mutations in the HRAS, PIK3CA, PIK3R1, MYC, and CCND3 genes. The final diagnosis was reported as melanocytic medullary thyroid carcinoma with high grade transformation and loss of epithelial and neuroendocrine expression.     

Bizarre parosteal osteochondromatous proliferation (Nora's lesion): a retrospective study of 12 cases, 2 arising in long bones

Twelve cases of bizarre parosteal osteochondromatous proliferation (BPOP), also known as Nora's lesion, are reported. Ten lesions were located in the small bones of the hands, and 2 were located in long bones (femur and proximal tibia). Patient age ranged from 12 to 63 years (average, 30.3 years). Radiography of the lesions in the hand bones showed calcific masses attached to the underlying cortex, without interruption of the latter. The long bone lesions revealed unusual findings. In the femur, BPOP presented with extensive cortical destruction and was suggestive of a malignant lesion. This presentation has not been described to date. In the tibia, the lesion was located in the soft tissue without cortical attachment. This type of BPOP probably represents an immature lesion that over time will mature to solid cortical attachment. On histologic examination, all lesions demonstrated 3 distinct components with variable degrees of representation: (1) hypercellular cartilage with calcification and ossification, with the calcified cartilage having a characteristic basophilic tinctorial quality; (2) cancellous bone undergoing maturation; and (3) spindle cell stroma without cytologic atypia. In 1 case with a long-standing history, the cartilaginous component was minimal. BPOP, together with florid reactive periostitis and turret exostosis, may represent different stages in the development of a posttraumatic proliferative process. BPOP apparently arises from the periosteal tissues through a process of cartilaginous metaplasia.     

Tumor-to-tumor metastasis: Hepatocellular carcinoma metastatic to parathyroid adenoma

Tumor‐to‐tumor metastasis is a rare, but well‐recognized phenomenon. Here, we report a unique case of hepatocellular carcinoma (HCC) metastatic to parathyroid adenoma. A 53‐year‐old‐man with a history of HCC presented with hypercalcemia. It was found that he also had hyperparathyroidism with a hypoechoic mass in the lower portion of the right thyroid gland area. The patient underwent parathyroidectomy, and a mass measuring 1.5 × 1.0 cm was detected. Microscopic and immunohistochemical examination disclosed parathyroid adenoma with a focus of metastatic HCC. For the patient, it was the only distant metastasis revealed until the last follow‐up. To the best of our knowledge, this is the first reported case of tumor‐to‐tumor metastasis in which HCC is the donor.     

不同类型血管蒂胫骨皮瓣治疗四肢骨不连

目的探讨采用不同类型血管为蒂胫骨皮瓣修复四肢骨不连的临床效果。方法 2004年6月至2018年10月,对41例四肢不同类型骨不连或伴有皮肤软组织缺损者,分别采用以单纯胫后血管束为蒂的桥式交叉胫骨皮瓣5例,以胫后动脉穿支为蒂的桥式交叉胫骨皮瓣修复9例、游离移植27例。切取胫骨瓣长度为1.0～8.0 cm,切取皮瓣面积为2.5 cm×2.0 cm～8.0 cm×25.0 cm。术后对所有患者均进行常规X线片随访,直至骨质愈合。结果术后对所有患者均进行随访。胫骨骨皮瓣全部成活,随访3～24个月,骨不连、骨缺损修复后,骨愈合良好,3～8个月骨折线消失。其中,27例皮瓣与修复区周围组织基本相平,14例有不同程度臃肿,经二期去脂整形手术后,也与周围组织平齐。19例掌骨或指骨骨不连的患者,术后按照中华医学会手外科学分会上肢部分功能评定试用标准中TAM系统评定法进行关节功能评定:优11例,良6例,可2例。22例胫骨骨不连的患者,术后恢复负重行走功能。结论三种不同类型血管为蒂胫骨皮瓣修复四肢骨不连均取得很好的治疗效果,其各有优缺点,又相互补充,是一类较为可行的骨不连修复方法。     

Exceptionally large solitary fibrous tumor arising from the cheek: an immunohistochemical and ultrastructural study with a review of the literature

Solitary fibrous tumor (SFT) is a rare mass-forming soft tissue tumor that occurs most commonly in the pleura, but has been described in various extrathoracic sites. Extrapleural manifestation of SFT, particularly in the head and neck region, is rare. The most common extrapleural site is the oral cavity; these tumors have also been described in the orbit, nasopharynx, paranasal sinuses, salivary glands, and larynx. We report an extremely rare case of a SFT in the subcutaneous region of the cheek. This tumor in the left cheek was a large firm mass, approximately 8.5 cm × 6 cm in size and was successfully treated by surgical resection. Immunohistochemistry revealed reactivity for vimentin, CD34, and bcl-2, but no staining for cytokeratin, epithelial membrane antigen, S-100, desmin, caldesmon, actin, α-smooth muscle actin, CD117, and CD99. Immunohistochemical study is the key to establish a definitive diagnosis of SFT, and ultrastructural study is also useful for making an accurate diagnosis. The patient recovered uneventfully without evidence of tumor recurrence 2 years after surgery.