青年外阴恶性间叶瘤1例并文献复习

恶性间叶瘤指由两种或两种以上互不相关的间叶成分构成的恶性肿瘤,临床罕见。国内外均有少量病例报道,多发生在中老年,男性多见,女性青年外阴部发病更为罕见。恶性间叶瘤好发于四肢,术前诊断困难,确诊需要依靠病理检查。肿瘤恶性程度高,术后复发率高,无临床特异性,又因影像学特征性表现少而不被引起重视。本文报道一例经病理证实的外阴恶性间叶瘤。     

An autopsy case of malignant mesenchymoma of the diaphragm

An autopsy case of a huge tumor in the right thorax is reported. A 71-year-old man complained of dyspnea and died after three months of hospitalization. The tumor, consisting of a circumscribed mass measuring 24 X 24 X 8 cm and weight 3.7 kg, originated from the right diaphragm and had no metastasis. Histologically, the tumor comprised multiple mesenchymal elements of fibrosarcoma, rhabdomyosarcoma, osteosarcoma and chondrosarcoma, and there was no distinct border between these different components. This tumor fulfills the criterion of malignant mesenchymoma (Stout). The histogenesis of the tumor is discussed with a survey of literature.     

Giant malignant mesenchymoma of the spermatic cord with bidirectional differentiation.

BACKGROUND: Spermatic cord neoplasms are a rare tumor entity and, moreover, of benign behavior. Malignant tumors of the spermatic cord are mostly of mesenchymal origin. We present the unusual case of a giant malignant mesenchymoma of the spermatic cord with bidirectional differentiation into a liposarcoma and a leiomyosarcoma. CASE REPORT: A 84-year-old male patient presented with a scrotal mass on the left side which was observed growing since 1 year and misdiagnosed as scrotal hernia or testicular hydrocele. Ultrasound and computed tomography demonstrated a solid tumor suggesting a spermatic cord tumor. The patient underwent hemiscrotectomy, and the histological examination of the 2,500-gram specimen revealed a malignant mesenchymoma originating from the spermatic cord with two distinct histopathological compartments of liposarcoma and leiomyosarcoma. Because an adjuvant therapy protocol is of questionable effect and because of the patient's age no further therapy was applied. The patient was closely followed and is now, 5 years after surgery, still free of disease. CONCLUSION: Even in older patients, scrotal masses should be considered malignant tumors as long as no benign diagnosis has been proven. Although malignant mesenchymomas are rare tumors with poor prognosis, in selected cases even large tumor masses, as presented, can be cured by surgery.     

Ganglioneuroblastoma associated with malignant mesenchymoma.

A case of ganglioneuroblastoma associated with malignant mesenchymoma is reported. The tumor originated from the retroperitoneum and was comprised not only of neuroblastoma and ganglioneuroma, but also of typical rhabdomyosarcoma, liposarcoma, undifferentiated mesenchymoma, as well as cartilaginous tissue. No distinct border existed between thses different components. The histogenesis of the tumor is discussed. We suggest that the tumor derived from a remnant of neural crest (ectomesenchyme).     

Malignant mesenchymoma of the bladder

A case of malignant mesenchymoma of the bladder, containing fibroleiomyomatous, myxomatous, osteoid, and cartilaginous components is presented. The primary pedunculated tumor measuring 18 X 10 X 9 cm and weighing 934 g, arose from the anterior wall of the bladder and was treated by partial cystectomy. Six months after surgery, cystoscopy revealed a recurrent tumor situated at the dome of the bladder. The patient received surgery, chemotherapy, and irradiation for recurrent tumor, but died of the disease 21 months after the first operation. Autopsy revealed extensive local recurrence with wide-spread metastases of malignant mesenchymoma of the bladder and an incidental finding of coexisting clear cell carcinoma in the right kidney (pTl, NO, MO). There have been no previous reports of association of malignant mesenchymoma of the urinary bladder and renal cell carcinoma.     

Mesenchymoma: A review of literature and report of two cases

Both benign and malignant varieties of the mesenchymoma have been described. Previously reported cases of mesenchymoma in the head and neck are rare. Two cases of mesenchymoma occurring intraorally are reported here. In each case local excision has effected apparent cure, for periods of 12 yr and 8 mo, respectively. Local excision is advocated as the primary treatment for such lesions occurring in the oral cavity. Although recurrences in areas other than the oral cavity tend to undergo malignant changes, this phenomenon has not been demonstrated in lesions occurring in oral tissues.     

Benign cysts of the parotid gland

Although sarcoma occurring in the true retroperitoneum is not uncommon, sarcoma in the retrorectal space is unique. We report a patient with a malignant mesenchymoma, a rare soft tissue tumor, in the retrorectal space. Complete resection was accomplished through a combined Kraske's posterior and midline anterior approach. The rectum had not been invaded by the mass. Survival of patients with malignant mesenchymoma does not exceed 50% in most reports, and the role of adjuvant chemotherapy or radiation has yet to be defined. Its attendant morbidity must be weighed against an uncertain prognosis.     

Malignant Mesenchymoma of the Liver with Hypercalcemia Report of a Case

A case of malignant mesenchymoma of the liver is reported. Thediagnosis was confirmed on the surgical specimen. The patientshowed marked hypercalcemia. Parathyroid hormone assay was negative.     

A rare case of benign mesenchymoma of the breast in a man.

The term 'benign mesenchymoma' was first used by Stout in 1948 and has since been widely adopted to describe benign tumours made up of a mixture of mesenchymal tissues which had previously been called by many names, such as hamartoma and angiolipoma. This tumour is most commonly found in the renal and perirenal tissue. Benign mesenchymomas arising in the breast are extremely rare. We present, to our knowledge, the first reported case of benign mesenchymoma in a male breast. The clinical presentation, course and treatment of the patient are discussed.     

Cardiac tumors of mice

Of the 7 primary tumors of the mouse heart, accessed at the Registry of Experimental Cancers, 3 were mesenchymal cell sarcomas, 3 were hemangioendotheliomas, and 1 was a mesenchymoma. The mesenchymal cell sarcomas metastasized via the bloodstream to lungs, kidneys, and spleen. Five of the tumors arose in mice treated with ionizing radiation. Previously, only 3 primary cardiac tumors of mice have been reported. Tumors of the heart as part of multifocal neoplasms of a single histologic type were observed in 3 mice; 2 of these tumors were type B reticulum cell sarcomas, and 1 was a reticulum cell sarcoma not otherwise specified. Of the metastatic tumors of the heart, 12 were from alveologenic carcinoma, squamous cell carcinoma, or carcinosarcoma of the lung, 4 were from sarcoma of the subcutaneous soft tissue, and 1 each was from a subcutaneous transplant of a malignant schwannoma and from an alveologenic tumor of the lung.     

Extraskeletal osteosarcoma of penis: A case report

Extraskeletal osteosarcoma (EOS) is rare and commonly arises in the retroperitoneum, limbs, head and neck. There is no significant difference between EOS and other malignant tumors in soft tissue. Localized pain and swelling are the common presenting symptoms. Clinical diagnosis of EOS is difficult, imaging techniques may be helpful and careful, and the histopathological analysis is necessary. The common histological variants of EOS include: osteoblastoma, chondroblastoma, and fibroblastoma, and other unusual subtypes were reported occasionally. It should be distinguished with myositis ossificans, malignant mesenchymoma, giant cell tumor and parosteal osteosarcoma. We present an EOS arising in the penis. The primary site and histological category of the tumor were extremely rare. We hope the case will be helpful to the recognition of clinical signs, iconography and histopathology of EOS.     

Malignant mesenchymoma. Case report with electron microscopic study.

A malignant tumor from the thigh of a 65-year-old man was examined by light and electron microscopy. The tumor was diagnosed by light microscopy as malignant mesenchymoma with prevalence of leiomyosarcomatous or liposarcomatous components, but electron microscopic study revealed that the basic cell was similar to chondroblastic or osteoblastic cells. However, a precise ultrastructural classification of the tumor cells was not possible, since the majority of the cells had mixed features. The histogenesis, differentiation, and relation to prognosis are discussed.     

Nonlymphoid mesenchymal tumors of the parotid gland

Salivary gland tumors are uncommon and most of them are of epithelial origin. Mesenchymal tumors affecting the parotid are extremely rare, and we present a series of 19 cases. All parotid tumors (600 cases) treated at the Department of Head and Neck Surgery from A.C. Camargo Hospital, Brazil from 1953 to 2003 were reviewed and 19 cases of nonlymphoid mesenchymal origin were selected. The histological characteristics were reviewed and clinical features were obtained from the medical charts. 15 out of 19 were benign tumors, including 5 lymphangiomas, 5 neurofibromas, and one case each of schwannoma, lipoma, solitary fibrous tumor, meningioma and giant cell tumor. Four malignant tumors were classified as rhabdomyosarcoma, fibrosarcoma, Langerhans cell histiocytosis and endodermal sinus tumor. From the malignant cases, only the patient with fibrosarcoma died due the tumor, the other three are alive with no signs of recurrence. In our series of 600 cases of parotid gland tumors, nonlymphoid mesenchymal tumors corresponded to 3.16% (19 cases; 15 benign and 4 malignant). All cases were treated by surgery with no recurrences, except one case of fibrosarcoma whose patient died of distant metastasis.     

Malignant rhabdoid tumor of the pelvis

An extrarenal malignant rhabdoid tumor (MRT) of the pelvis in a 14-year-old girl is described. The location of the tumor and age of the patient appear to be unique. The neoplasm is histologically and ultrastructurally identical to malignant rhabdoid tumor of the kidney seen in infants and very young children. The tumor had an aggressive clinical course, and the patient died with disseminated disease 13 months after diagnosis. The authors review previous reports of extrarenal MRT, discuss the occurrence of cytoplasmic filamentous inclusions, and contrast MRT with other pelvic sarcomas. Although the exact histogenesis is unknown, ultrastructural and immunohistochemical data suggest a mesenchymal origin.     

Malignant mesenchymoma of the mesocolon

A rare case of malignant mesenchymoma in a 58-year-old woman is described. The tumor grew rapidly for a month and was located in the mesosigmoid. The resected tumor measured 25 X 20 X 20 cm and weighed 5,000 g. It metastasized into the great omentum within four months after operation. Histologically, this tumor was composed of undifferentiated sarcomatous elements, but in some parts revealed patterns of differentiation, including leiomyosarcoma, chondrosarcoma and osteosarcoma. In areas of osteosarcoma, the bone tissue varied from immature to mature. The histology of the recurrent tumors was similar to that of the first tumor. We considered that undifferentiated sarcomatous elements contained pluripotential cells which could differentiate multiple, mature malignant mesenchymes.     

Malignant neoplasm of mixed mesenchymal and neuroepithelial origin (ectomesenchymoma) of thigh

A 49-year-old man had a malignant soft tissue tumor of the right thigh with metastasis to the femoral region and lower quadrant of the anterior abdominal wall on the right side and the left supraclavicular lymph nodes. The neoplasm showed features of chondrosarcoma and primitive neuroectodermal tumor (combined neuroblastoma, ependymoma, astrocytoma, and oligodendroglioma). The gliomatous part of the mixed tumor was confirmed by identification of the glial fibrillary acidic protein (GFAP). The diverse cellular population suggests a tumor origin from the ectomesenchymal remnant of the neural crest. The mesenchymal component of the neural crest would differentiate into the chondrosarcoma and the neuroectodermal component into the primitive neuroectodermal neoplasm. These various neoplastic elements, then, would form a neoplasm of mixed mesenchymal and neuroepithelial origin or an ectomesenchymoma.     

Malignant pediatric brain tumor of primitive small round cell proliferation with bland-looking mesenchymal spindle cell elements

It is often difficult to classify rare malignant pediatric mixed brain tumors composed of mesenchymal elements. A 6-year-old boy presented to our hospital with a left frontal massive tumor manifesting as complaints of rapidly progressive right hemiparesis and motor aphasia over 2 weeks. Computed tomography showed a left frontal mass with thick calcification. Magnetic resonance imaging revealed an enhanced lesion with perifocal edema and mass effect. Total removal of the tumor was performed. Histological examination of the resected tumor revealed an anaplastic malignant small round cell component with a bland-looking mesenchymal spindle cell component. The patient was treated with irradiation to the whole craniospinal axis and a boost to the tumor bed, followed by chemotherapy consisting of ifosfamide, cisplatin, and etoposide, resulting in good control without local recurrence or metastasis at 2 years. A combined malignant tumor composed of ectodermal and mesenchymal components is generally named malignant ectomesenchymoma (MEM). The more malignant part of MEM is the mesenchymal component in most cases. In the present case, the more malignant component was not the mesenchymal component, but the small round cells.     

Sarcoma Following Irradiation for Breast Cancer: Report of Three Unusual Cases Including One Malignant Mesenchymoma of Bone

Three cases of sarcoma developing after irradiation for breast cancer are reported. A malignant mesenchymoma in the sternum—a combination of osteogenic sarcoma and rhabdomyosarcoma—is the first documented case of its kind occurring after radiation therapy. Of the other two tumors one was an extraskeletal osteogenic sarcoma in the soft tissues of the thoracic wall and one a rhabdomyosarcoma in the axilla.     

Primary pedunculated fibrosarcoma of the peritoneum: A case report

Fibrosarcoma is a primitive, mesenchymal tumor frequently arising from superficially located soft tissues. A case of a primary intraperitoneal, pedunculated fibrosarcoma is reported, initially presenting as an abdominal mass protruding into a large incisional hernia of the anterior abdominal wall. Despite total tumor resection, multiple intraperitoneal tumor nodules of varying sizes were found and resected six months and one year later. The patient died 18 months after the first surgical intervention. This case illustrates the highly malignant behavior and poor prognosis of fibrosarcomas of the peritoneum.     

肝肉瘤样癌1例并文献复习

目的：探讨肝肉瘤样癌患者的临床及病理学特征、诊断、鉴别诊断、治疗和预后。方法：结合我院收治的肝肉瘤样癌1例临床资料,分析国内外文献报道的该病临床特点、影像学表现、病理学特点,治疗和预后。结果：大部分该病患者均有腹痛和发热的临床表现,病理学上肿瘤都包含明确的恶性上皮性成分和梭形细胞肉瘤样成分且上皮和间叶性两种标记物表达阳性,愈后差。结论：肝肉瘤样癌是一种罕见的恶性肿瘤,恶性程度高,浸润性强,病因不明,目前无有效治疗,预后很差。