Histological and clinical characteristics of malignant giant cell tumor of bone

Malignant giant cell tumors of bone (MGCTB) are rare, and the diagnosis can be difficult due to the occurrence of a variety of malignant tumors containing giant cells. To better understand its clinicopathological features, we have reviewed our experience with 17 cases of MGCTB. Five cases were primary malignant giant cell tumor of bone (PMGCTB), and 12 cases were giant cell tumors of bone initially diagnosed as benign but malignant in a recurrent lesion (secondary MGCTB, SMGCTB). The patients included six women and 11 men (age ranged from 17 to 52 years; mean, 30.5 years). The tumor arose in the femur (six cases), the tibia (seven cases), the humerus (three cases), and the fibula (one case). Microscopically, PMGCTB showed both conventional giant cell tumor and malignant sarcoma features. SMGCTB were initially diagnosed as conventional giant cell tumor of bone, the recurrent lesion showing malignant features. Histologically, the malignant components included osteosarcoma (11 cases), undifferentiated high-grade pleomorphic sarcoma (two cases), and fibrosarcoma (four cases). SMGCTB cases showed strong expression of p53. Follow-up information revealed that four patients died of lung metastasis, two patients are alive with lung metastases, and 11 patients are alive without tumor. MGCTB should be considered as a high-grade sarcoma. It must be distinguished from GCTB and other malignant tumors containing giant cells. p53 might play a role in the malignant transformation of GCTB.     

Biology of metastasizing ameloblastoma

The present report of a malignant metastasizing ameloblastoma and a critical review of literature was undertaken in an attempt to better understand the biological potential and behavior of this rare tumor and thus to facilitate its clinical management. Most of the 26 patients with a proven malignant ameloblastoma including the present case had developed multiple recurrences. The lung was the most frequent metastatic site (88%) followed by regional lymph nodes (27%). Furthermore metastases were observed in some cases in the bone, brain, kidney, small intestine and liver. The interval between diagnosis of tumor and manifestation of metastases was long with a median of 11.1 years. The average survival time was 13.1 years. By contrast, the interval between diagnosis of metastatic disease and death was relatively short (median: 2.6 years). The histologic and cytologic pattern of malignant ameloblastoma and of its metastases was not significantly different from that of non-metastatic ameloblastoma. Because of the lack of morphological criteria of malignancy the biological behavior of ameloblastomas cannot be predicted. It is difficult to be certain which factors are important in the delayed induction of metastases. It is suspected that ameloblastomas possess an inherent low grade malignancy which is stimulated by multiple recurrences. It is further assumed that the metastatic tumor cells have a slow growth rate resulting in late clinical manifestation of metastases. When lung metastases occur we recommend their surgical removal in order to prolong live expectancy or even to obtain a curative effect.     

Epidemiology of bone tumors in Mexico City: retrospective clinicopathologic study of 566 patients at a referral institution

A retrospective analysis of all bone tumors accessioned at a large referral center (Instituto Nacional de Rehabilitacion) in Mexico City between 2000 and 2005 is presented. A total of 6216 biopsies and surgical resection specimens were reviewed during this period, of which 566 corresponded to bone tumors. Benign bone tumors accounted for 71.6% of cases and malignant bone tumors for 28.4%. The tumors affected men in 53.7% of cases and women in 46.3% of cases, with an average age at presentation of 25 years. The femur was the most common location of the tumors (39.9%), followed by the tibia (17.7%) and humerus (11.8%). The commonest malignant bone tumors were osteosarcoma (46.6%) and chondrosarcoma (8.7%). Of malignant bone tumors, 18.6% corresponded to metastases of carcinomas from internal organs and 8.1% were multiple myeloma. The most common benign bone tumor was osteochondroma (43.7%) followed by giant cell tumor of bone (14.6%) and enchondroma (10.1%). The age distribution showed a peak in children and adolescents comprised predominantly of benign lesions and a second peak in young adults that corresponded to malignant bone tumors (principally osteosarcoma). Malignant bone tumors most often involved the femur, vertebra, and tibia. Our results parallel the findings previously reported in the world literature and show a similar distribution and epidemiology as in other developed and underdeveloped countries. Geographic location does not appear to represent a risk factor for any particular type of bone tumor and does not affect the age distribution, location, or histopathologic type of the lesions.     

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: Clinicopathologic features of 5 cases

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal tumor of intermediate malignant potential with characteristic clinicopathologic and genetic features. Although bone involvement accompanies nearly one-fourth of reported cases of soft tissue PHEs, primary intraosseous PHE is rare. Herein, we report five cases of primary intraosseous PHEs. Male to female ratio was 4:1, with an average age of 28 years (age range, 5–44 years). Radiologically, tumors presented as lytic lesions in the proximal femur (two), diaphysis of the tibia (one), distal radius (one) and vertebrae (one). Multifocal lesions were observed in four cases. Histopathologic examination revealed plump spindle cells and prominent nucleoli. New bone formation was noted in three cases. Immunohistochemically, all tumors were positive for CD31 and negative for CD34. Pan Cytokeratin (CK) (AE1/3) was positively expressed in all, except a single tumor, in which CK7 and Cam5.2 were expressed. INI1/SMARCB1 was completely retained in all tumors. A single patient underwent surgical resection. During follow-up, two cases showed no evidence of disease within two and five years, respectively. Differential diagnosis of a PHE of bone includes osteoblastoma, epithelioid angiosarcoma, metastatic carcinoma, metastatic rhabdomyosarcoma, and epithelioid sarcoma. Caution must be exercised as pan CK (AE1/3) might not be expressed; therefore, the use of other cytokeratins, such as Cam5.2 is recommended. Awareness of such an entity in bone is the key to the diagnosis.     

Epithelial tumors of the lacrimal glands: a clinicopathologic study.

We report the clinicopathologic features of epithelial tumors of the lacrimal gland apparatus, which are rare and therefore represent a major challenge for diagnosis and treatment. Histologic material from 22 lesions was studied by light microscopy, histochemistry, and immunohistochemistry. A comparison with major and minor salivary gland tumors was performed to analyze the relative distribution of these tumors and to establish whether salivary glands and lacrimal gland tumors are similar or different in their pathologic appearance and clinical behavior. There were three benign pleomorphic adenomas and 19 malignant tumors. The gender distribution was equal. The ages of the patients ranged from 10 to 73 years (mean age, 46 years). Among the malignant tumors, adenoid cystic carcinoma was the most common (nine cases), followed by mucoepidermoid carcinoma (three cases). There were two cases each of malignant mixed tumor and adenocarcinoma. All mucoepidermoid carcinomas and the adenocarcinomas were histologically high grade. There also was one case each of salivary duct carcinoma, spindle cell carcinoma, and oncocytic adenocarcinoma. Of 14 patients in whom clinical follow-up was available, seven had distant metastases and four died of their disease. The only case occurring in a child was an adenoid cystic carcinoma that recurred locally after 14 years. The clinical and pathologic features of lacrimal gland tumors resemble those lesions that arise in the intraoral minor salivary glands. The greater relative proportion of malignant cases in this series probably reflects a selection bias.     

Poorly differentiated carcinoma of the thyroid retrospective clinical and morphologic evaluation

Five thyroid carcinomas showing clinically aggressive biologic behavior were retrospectively reviewed to evaluate the possible presence of morphologic differences from conventional thyroid carcinomas. They were originally diagnosed as follicular carcinomas, medullary carcinoma, papillary carcinoma, and mixed papillary and medullary carcinoma. There were three males and two females. The age at the time of initial diagnosis ranged from 36 years to 67 years (mean 56 years). The size of the tumor varied from 4.5cm to 10cm (mean diameter 7cm). One patient died of brain metastasis four years after the initial therapy and the other four patients are still alive with local recurrences and/or metastases to bone, spinal cord, lung, and buttock. Histologically these lesions are categorized into two different groups: insular variant and columnar cell variant. Insular variant was characterized by well-defined nests (insulae) that are composed of small, uniform cells, frequent tumor necrosis, and hyalinization of the stroma. Columnar cell variant was characterized by tall columnar cells with marked nuclear statification. All five cases stained positively for thyroglobulin and negatively for calcitonin. With the above clinical and histopathological findings, we interpreted these lesions as a poorly differentiated carcinoma, biologically in an intermediate position between well differentiated and anaplastic carcinomas. The rapid and often fatal outcome associated with these variants of poorly differentiated carcinoma warrants aggressive treatment at the time of diagnosis.     

Orthopaedic implant-related sarcoma: a study of twelve cases.

Sarcoma developing in association with a metallic orthopaedic prosthesis or hardware is an uncommon, but well recognized complication. We review 12 cases of sarcomas arising in bone or soft tissue at the site of orthopaedic hardware or a prosthetic joint. Nine patients were male, and three were female. Their ages ranged from 18 to 85 (mean 55) years at the time of diagnosis of the malignancy. Five patients had undergone hip arthroplasty for degenerative joint disease, four had been treated with intramedullary nail placement for fracture, two had staples placed for fixation of osteotomy, and one had hardware placed for fracture fixation followed years later by a hip arthroplasty. The time interval between the placement of hardware and diagnosis of sarcoma was known in 11 cases and ranged from 2.5 to 33 (mean 11) years. The patients presented with pain, swelling, or loosening of hardware and were found to have a destructive bone or soft tissue mass on radiography. Two sarcomas were located primarily in the soft tissue and 10 in bone. Seven patients developed osteosarcoma, four malignant fibrous histiocytoma, and one a malignant peripheral nerve sheath tumor. All sarcomas were high grade. Three patients had metastatic disease at the time of diagnosis. Follow-up was available on eight patients: five patients died of disease 2 months to 8 years (mean 26 months) after diagnosis; two patients died without evidence of disease 7 and 30 months after diagnosis; and one patient is alive and free of disease 8 years after diagnosis. Sarcomas that occur adjacent to orthopaedic prostheses or hardware are of varied types, but are usually osteosarcoma or malignant fibrous histiocytoma. They behave aggressively and frequently metastasize. Clinically, they should be distinguished from non-neoplastic reactions associated with implants, such as infection and a reaction to prosthetic wear debris.     

Prognostic value of immunocytologic detection of bone marrow metastases in neuroblastoma

BACKGROUND. Morphologic evaluation of bone marrow for neuroblastoma cells is a routine and important component of clinical staging. Specific immunostaining of malignant cells with monoclonal antibodies should be more sensitive, however, and may improve the detection of metastases and provide additional prognostic information. METHODS. We looked for tumor cells in bone marrow from 197 patients with newly diagnosed neuroblastoma, using immunoperoxidase staining with monoclonal antibodies (immunocytologic analysis) and examination of smears and specimens obtained by trephine biopsy (conventional analysis). RESULTS. Routine smears and trephine-biopsy specimens were positive for tumor cells in 46 percent of the patients, whereas 67 percent were positive on immunocytologic analysis (P less than 0.0001). Immunocytologic analysis detected bone marrow metastases in 34 percent of patients considered to have only localized or regional disease (Stage I, II, or III). It also identified tumor cells that were not detected by conventional analysis in patients with widespread disease (Stage IV or IVS). Tumor content, as determined by immunocytologic analysis, predicted clinical outcome in relation to the age of the patient at diagnosis. Patients with Stage II or III disease diagnosed after one year of age who did not have occult marrow metastases did well, whereas those with metastases did poorly (P = 0.006). Patients in whom Stage IV disease was diagnosed before they were one year of age did well if bone marrow metastases were few or absent, but had poor survival if the marrow contained more than 0.02 percent tumor cells (P = 0.03). CONCLUSIONS. Immunocytologic analysis of bone marrow aspirates is more sensitive than conventional analysis in detecting tumor cells and provides prognostic information. The relations among marrow metastases, age at diagnosis, and clinical outcome illustrate the biologic heterogeneity of neuroblastoma.     

Malignant epithelial tumors and hematologic malignancy

The aim of this study was to analyze the value of cytology in differentiation between malignant epithelial tumor metastases and hematologic malignancy. The follow-up of ten (10) patients who underwent diagnosis and treatment of two malignant diseases, i.e. carcinoma and hematologic malignancy, was performed in the 2000-2005 period. The median of age of our patients was 72 years (range: 49-79). Cytological examination included epithelial tumors, lymph nodes and bone marrow standard Pappenheim and immunocytochemically stained smears. Carcinoma was initially diagnosed in 40% (4/10) patients and hematologic malignancy in 50% (5/10) patients, while both diseases co-occurred in one patient (1/10). Most of hematologic malignancy cases (4/10) were diagnosed as lymphoma. Multiple myeloma was diagnosed in 3 out of 10 patients (30%). Individual cases of acute myeloblastic leukemia, chronic lymphocytic leukemia, and chronic myeloid leukemia were diagnosed in the remaining three patients. Most carcinomas were breast cancer (8/10), while prostate and thyroid gland cancer were diagnosed each in one patient, respectively.     

Metastatic tumors to the female breast. An autopsy study of 12 cases

Twelve cases of metastatic tumors to the female breast are reported. Ten of them were incidentally discovered at autopsy; two cases were diagnosed ante mortem but one was misinterpreted as primary. The patients' mean age was 58 years. The metastasizing tumors included cutaneous malignant melanoma (four cases); ovarian, renal and gastric adenocarcinoma (two cases each); and individual cases of pulmonary and pancreatic carcinoma. The patients with melanoma were younger than the others (49.7 vs. 62.7 years). The results of the present study indicate that breast metastases, although rare, are not exceptional especially in large autopsy series. Their recognition in surgical material would result in more adequate treatment against the primary tumor, thereby avoiding unnecessary radical surgery to the breast.     

Colic metastases of alveolar soft-part sarcoma: a case report and review of the literature

Alveolar soft-part sarcoma is a rare soft tissue malignant tumor that commonly arises in skeletal muscles or musculofascial planes in children and young adults. Metastases of lung, brain, and bone are often present at the time of diagnosis or appear late in the course of disease. We present the first case, to our knowledge, of colic metastases in a 43-year-old woman who had a leg primary tumor more than 15 years ago and multiple lung and brain metastases. The treatment of these two caecal metastases, revealed by an anemia, consisted in laparoscopic right colectomy.     

骨的恶性巨细胞瘤临床病理观察

目的 探讨骨的恶性巨细胞瘤临床病理学特征及其诊断和鉴别诊断.方法 对13例恶性巨细胞瘤的临床及病理学资料进行回顾性分析.结果 13例恶性巨细胞瘤中原发性恶性巨细胞瘤6例,发病年龄21-71岁,平均年龄39.5岁.复发后继发性恶性巨细胞瘤7例,发病年龄27-52岁,平均年龄36.7岁.6例原发性恶性巨细胞瘤除可见到骨巨细胞瘤区域外,还可见到高度恶性的梭形细胞肉瘤区域,7例继发性恶性巨细胞瘤其原发性肿瘤均为骨巨细胞瘤,而复发性肿瘤则呈恶性纤维组织细胞瘤/未分化肉瘤形态.结论 诊断恶性巨细胞瘤时需将临床、影像及病理结合,并除外其他肉瘤如富含巨细胞的骨肉瘤、富含巨细胞的恶性纤维组织细胞瘤等.     

Clear cell sarcoma of the kidney: A clinicopathologic study of 21 patients with long-term follow-up evaluation

The clinical and pathologic features of 21 children with clear cell sarcoma of the kidney are presented. These cases were identified among 517 primary renal tumors collected from four pediatric institutions. Abdominal mass and hematuria were the usual presenting signs. The male to female ratio was 1.3 to 1. Although the mean age at diagnosis was 3 years, the sarcomas were diagnosed earlier in boys (2 years, 7 months) than in girls (3 years, 7 months). Tumor size and weight may have accounted for this difference, since the average tumor weights were 899 g for boys and 635 g for girls. Right kidney locations predominated (14 cases). The most common site for metastasis was the skeleton (12 patients), and the skull was almost invariably involved (10 children). Bone metastases preceded the development of metastases elsewhere in nine patients, in five of whom the involvement was polyostotic. Of the 12 patients with osseous metastases, nine died within five years of diagnosis, and one died eight years and nine months after diagnosis; the remaining two patients were successfully treated with a combination of surgery, radiotherapy, and actinomycin D, vincristine, cyclophosphamide, and Adriamycin. Eight of the nine patients with lung metastases died within five years of diagnosis. Until substantial proof is found that clear cell sarcoma of the kidney is related to nephroblastoma, its identification as a non-Wilms' sarcoma would seem to be appropriate. This tumor is not the only sarcoma of the kidney, but it appears to be the most common renal sarcoma of childhood.     

卵巢肿瘤169例临床分析

目的：探讨卵巢肿瘤的临床特点,以期对其进行早期诊断、早期治疗并改善预后。方法：对我院5年间收治的卵巢肿瘤169例的临床资料进行回顾性分析．其中采用保守性手术治疗157例（92．90％）,根治性手术治疗9例（5．33％）,减瘤术治疗3例（1．78％）。结果：169例卵巢肿瘤患者中,良性、交界性及恶性肿瘤分别为155例（91．71％）、3例（1．78％）、11例（6．51％）。55岁及以上年龄组恶性肿瘤发病率14．29％,高于55岁以下组的4．48％（P〈0．05）。良性及交界性肿瘤治愈率为100％,恶性肿瘤11例中死亡4例（36．36％）：结论：卵巢肿瘤发病多较隐匿,定期作妇科检查及普查普治,对该病的早期诊断、治疗和改善预后有重要意义。     

足踝部骨与关节肿瘤及瘤样病变103例分析

目的研究足踝部骨与关节肿瘤及瘤样病变的发病规律。方法总结20年来齐鲁医院经病理确诊的足踩郜骨肿瘤及瘤样病变103例，结合文献分析。结果最高发病年龄集中于10～39岁组之间，男性多于女性，其比例为1．45：1。病变多何于跗骨，跖骨，趾骨。良性肿瘤及瘤样病变明显多十恶性肿瘤，其比例为6．38：1。良性肿爝67例，其中骨软骨瘤、软骨瘤最多见（占良件肿瘤的55．22％）；瘤样病变16例中，骨囊肿9例，最多见（占56125％）：恶性肿瘤13例，以滑膜肉瘤和软骨肉瘤最多见（分别有5例及3例）。结论足踩部骨肿瘤与瘤样病变良性多于恶性，病变多位于跗骨与跖骨。对足部恶性肿瘤的诊断应重视。     

Testicular metastases from solid tumors: an autopsy study

In a retrospective study of 738 consecutive autopsies of adult males with solid malignant neoplasms, five (0.68%) were shown to have metastatic deposits within the testis. These were metastases from bronchial carcinoma (three cases), melanoma (one case), and pancreatic endocrine carcinoma (one case). The mean age of the patients with solid tumors metastatic to the testis was 60 years (range, 32 to 83 years). The metastases from the solid tumors presented two patterns: destructive and/or focal interstitial. The destructive pattern was characterized by sheets of malignant cells that destroyed and replaced the seminiferous tubules. The interstitial pattern was characterized by tumor cells within the interstitium without involvement of the seminiferous tubules. All metastases showing the destructive pattern were macroscopically evident. Approximately 40% of the metastatic deposits were microscopic and showed a focal interstitial pattern. These cases are not easily identified unless a sufficient number of tissue blocks are sampled and carefully scrutinized. In 20% of the cases the metastatic deposits were bilateral. Improved diagnostic skills and treatment protocols in the last 11 years apparently have not significantly affected incidence, distribution, or the pattern of metastatic spread of solid tumors to the testis.     

原发性颅底软骨肉瘤九例的临床病理学分析

目的探讨原发性颅底软骨肉瘤的临床病理特点、免疫表型、分子特征及鉴别诊断。方法收集福建医科大学附属第一医院2006年1月至2019年6月9例原发性颅底软骨肉瘤的临床资料、影像学表现、大体及镜下形态、免疫表型及分子特征。结果9例患者中6例男性,3例女性,平均年龄47岁,中位年龄47岁。5例为WHOⅠ级肿瘤,4例为WHOⅡ级。镜下于软骨样或黏液样基质的背景中,见低-中等密度的肿瘤细胞呈结节状生长,细胞轻-中度异型,可见双核细胞,核分裂象罕见或偶见。免疫组织化学示肿瘤细胞S-100蛋白、波形蛋白、SOX9、D2-40阳性,Brachyury、细胞角蛋白(CK)、上皮细胞膜抗原(EMA)、CK8/18阴性,Ki-67阳性指数低(1%~5%)。分子检测4例呈IDH1 R132C位点突变。术后4例辅助放疗,随访1~59个月,8例存活。结论颅底软骨肉瘤是罕见的软骨来源的恶性肿瘤,预后好,其特征性的组织学表现结合免疫组织化学和分子检测有助于鉴别诊断。