右小腿多发性恶性血管球瘤一例

患者男,17岁。右膝疼痛1年,近一周加重于2005年12月就诊。患者自发病以来无发热、消瘦。体检：右侧胫骨中上段前外侧隆起,局部皮温略高,轻压痛。右侧胫、排骨正侧位X线示：软组织影像,胫、腓骨受累（图1）。     

Intraosseous glomus tumor in the sacrum. A case report

Primary intraosseous glomus tumor is rare and may occur in the medullary cavity of bone. A 22-year-old woman with a one-year history of spontaneous and persistent sacral pain was found to have an intraosseous glomus tumor in this unusual location. Curettage was followed by complete relief from pain. This case of glomus tumor originating in the sacrum is thought to be the first reported in the world literature.     

Symplastic glomus tumor: report of a challenging lesion with literature review

Glomus tumors are uncommon mesenchymal tumors whose cells closely resemble those of a normal glomus body, and are found most commonly in the hand. Recently, a symplastic form exhibiting marked nuclear atypia, in the absence of any other malignant features, has been described. To date, only 14 cases of symplastic glomus tumor have been reported in the literature; hence, very little information is available about its diagnosis, treatment, and biological behavior. The case reported here concerns a symplastic glomus tumor occurring in the right index finger of a 62-year-old woman. We reviewed the literature concerning previously reported cases, placing emphasis on the documented biological behavior, treatment, and demographic trend. Physicians must be aware of this morphological variation of glomus tumor to avoid the mistake of malignancy, which results in over-treatment of the patient.     

Carcinosarcoma of the colon: report of a rare tumor

Carcinosarcoma is a rare tumor that shows both epithelial and stromal malignant differentiation. Most reported cases of carcinosarcoma affect the female genital tract (and are called malignant mixed müllerian tumors), but there are also some isolated reports of cases affecting the lung and the head and neck area. Carcinosarcomas only rarely affect the gastrointestinal tract, mainly the esophagus. To the best of our knowledge, only eight cases of carcinosarcoma of the colon have been reported to date. For some lesions, the term 'sarcomatoid carcinoma' is preferred to 'carcinosarcoma', as both stromal and epithelial cells have shown cytokeratin expression on immunohistochemistry. The expression 'carcinosarcoma' should be applied only to those lesions, the stromal elements of which do not express epithelial markers. We report a new case of carcinosarcoma affecting the left colon. The most unique feature of this tumor is that it shows chondro-and osteosarcomatous differentiation, a feature that has been described previously in only one colonic carcinosarcoma. We discuss the histopathological and immunohistochemical features of this lesion as well as its possible histogenesis.     

Gastric glomus tumor

Gastric glomus tumors are rare neoplasms that may present with upper gastrointestinal symptoms, or may be discovered incidentally. They may mimic other tumors both grossly and microscopically. They are usually benign and, although the exceptional case with aggressive behavior cannot be reliably predicted, large size may be of prognostic importance. We describe a 3.8 cm gastric glomus tumor in a patient who also had adenocarcinoma arising in an adenoma of the rectum, and discuss the differential diagnosis and prognosis.     

Pulmonary glomus tumor

Glomus tumor (GT) is an infrequent but distinct neoplasm. Pulmonary GT is a rare neoplasm with only a few cases reported in the literature. These tumors are usually benign and, although rare, tumors with aggressive behavior have been reported. The tumor size, location, and histomorphological features may be useful in predicting tumor behavior. We present here a case of pulmonary GT that was initially diagnosed as a typical carcinoid tumor. The differential diagnosis as well as the recent classification of GTs is discussed along with a review of literature.     

Pulmonary glomus tumor

Glomus tumor, also known as glomangioma, is a neoplasm derived from cells of the neuromyoarterial glomus or glomus body. We report a case of glomus tumor of the lung arising in the left lower lobe, incidentally found in a patient who underwent right bilobectomy for a carcinoma localized in the right upper lobe.     

Primary glomus tumor of the liver: a rare case report and review of the literature

Glomus tumor is a rare neoplasm of the distal extremities. It occurs very rarely in the deep visceral organs such as stomach, lung, pancreas, ovary, and liver. Herein, we report a very rare case of glomus tumor of the liver in a 50-year-old woman presenting with abdominal mass which was diagnosed after surgery. The tumor was large and cystic; however, the morphology was similar to the ordinary soft-tissue glomus tumor. Tumor cells were reactive with CD34 and SMA. The patient's follow up failed to show any evidence of malignant behavior or tumor recurrence. This report is the third primary glomus tumor of the liver in the English literature.     

Malignant glomus tumor of the leg developed in the context of a superficial typical glomus tumor

A 41-year-old man presented with a 2-cm painful subcutaneous nodule in his right leg, which had been present for more than 10 years and was recently enlarging. Histologically, the tumor was composed of sheets and nests of cells with variable cytomorphology, including typical round/ovoid glomus cells with clear cytoplasm and well-defined borders, small cells, and spindle cells. Numerous medium to large vessels were present. Nodules with moderate to high cellularity, nuclear atypia, and frequent mitotic figures (42 per 50 high-power fields) were noted. Immunohistochemistry showed cytoplasmic and membranous expression of smooth-muscle actin, vimentin, and membranous expression of type IV collagen. Whereas superficiality, dimensions, and long-term follow-up may suggest classification as a symplastic or uncertain potential glomus tumor (GT), histological features and immunoprofile are indicative of malignant nodules developed in a typical GT. The follow-up has been negative for recurrence/metastases. A search of the literature revealed 17 cases of malignant GTs between 1995 and 2010, all fatal, of which 10 had skin as the primary site.     

Malignant glomus tumor of the lung

Primary malignant glomus tumors of the lung are extremely rare, and to our knowledge, only three cases have been described to date. We report one such case in a 53-year-old man who presented with a persistent dry cough. Chest computed tomography scans demonstrated an irregularly shaped mass in the right lower lobe of the lung. Many small nodules were distributed from the main tumor to the periphery, along with bronchovascular bundles. Right lower lobectomy was performed under the diagnosis of lung tumor. The tumor was located in the proximal portion of the right lower lobe and extended along the pulmonary arteries. Histological examination revealed a sheet-like proliferation of epithelioid glomus cells and fascicles of spindle cells. The presence of increased mitotic activity, tumor necrosis and prominent intravascular invasion suggested malignancy. The tumor cells were immunoreactive for vimentin, calponin, h-caldesmon, and alpha-smooth muscle actin, which indicated definitive smooth muscle differentiation. We believe that this is the fourth reported case of malignant glomus tumor of the lung.     

Intranasal pericytic tumors (glomus tumor and sinonasal hemangiopericytoma-like tumor): report of two cases with review of the literature

An intranasal glomus tumor and a sinonasal hemangiopericytoma-like tumor are reported. Both patients were elderly women suffering from nasal bleeding, and presented with a polypoid mass arising in the nasal septum. Microscopically, the glomus tumor displayed a proliferation of uniform rounded or cuboidal epithelioid cells arranged in sheets and interrupted by a rich vasculature with a characteristic configuration mimicking the normal glomus bodies, while the sinonasal hemangiopericytoma-like tumor featured a perivascular proliferation of spindle- to oval-shaped cells that were arranged in short fascicles. Both tumors shared immunohistochemical features supporting their myoid differentiation by the expression of vimentin, alpha-smooth muscle actin and muscle-specific actin, albeit with no immunoreaction to desmin. Both the intranasal glomus tumor and sinonasal hemangiopericytoma-like tumor are characterized by a perivascular growth pattern and myoid differentiation, having a close relation to the 'perivascular myomas', which was recently designated.     

Catecholamine secreting tumor of the glomus jugulare

Summary A case of endocrinologically active glomus jugulare is presented. The localization within the tumor of the catecholamine hyperproduction was demonstrated by selective venous sampling from the vena jugularis externa draining the tumor.The norepinephrine/epinephrine ratio was high and of the same order in blood, urine and cerebrospinal fluid. Bioassay showed high levels of intratumoral norepinephrine and epinephrine.The ways of treatment and their influence on the catecholamines are briefly discussed. In every phaeochromocytoma-like syndrome both the existence of catecholamine hyperproduction and the anatomical site of this hypersecretion have to be precisely documented in order to define the correct treatment to be applied.     

Malignant glomus tumor of the urinary bladder

We present a case of a malignant glomus tumor arising in the urinary bladder of a 57-year-old woman with metastatic pulmonary nodules who died 2 months later. Pathologically and clinically confirmed malignant glomus tumors are exceedingly rare, especially those that arise in the visceral organs. The present case retained its architectural similarity to a benign glomus tumor and consisted of sheets of highly malignant round cells showing cytoplasmic positivity for smooth muscle actin. On reticulin histochemical staining, we found that reticulum fibrils surrounded individual tumor cells, suggesting cellular investment by basement membrane. We discuss the concept of malignant glomus tumors and emphasize the criteria that distinguish them from other malignant tumors.     

Adenomatoid tumor of the pleura. Case report

We report a case of an adenomatoid tumor of particular location within the pleura, incidentally discovered on a pulmonary lobectomy specimen after surgical resection of a pulmonary squamous cell carcinoma. This adenomatoid tumor appeared as a unique pleural mass located away from the primary carcinoma and consisted of a cellular proliferation organised in tubes and sheets. Adenomatoid tumors are considered as benign tumors of mesothelial nature. Their morphological and immunohistochemical features in association with their location to the pleura, warrant a precise analysis to eliminate malignant tumours such as malignant mesothelioma or metastatic adenocarcinoma.     

Benign glomus tumor of the superior posterior mediastinum

An unusual location of a benign glomus tumour, outside of the constantly located regions, e.g. in the subungual location or deeply sited in extremities, was diagnosed in a 56-year-old white female in her posterior upper mediastinum. The single similar case report was published before the era of electron microscopy and immunohistochemistry and single cases of atypical and malignant forms in this unusual location were published only recently. The tumour measuring 5 x 4 x 2 centimeters has caused cough and was associated with occasional righ-sided chest pain. Its rich vascular supply has caused intensive intraoperative bleeding. The postoperative course was uneventful and the patient is free of neoplastic disease or symptoms six years after surgery. Numerous mast cells present within the tumour's interstices must be considered in relation to the possible pathogenesis of the up to now unexplained pain in glomus tumours.     

Glomangiomyoma (glomus tumor) of the kidney

We report herein a case of glomus tumor arising in the kidney of a 55-year-old woman, which was found incidentally on a computed tomographic scan. Partial nephrectomy revealed a 2-cm encapsulated mass that was architecturally similar to glomus tumor. Immunohistochemistry showed positivity of tumor cells for vimentin and smooth muscle actin. On electron microscopy, cytoplasmic thin filaments and dense bodies were seen, confirming the smooth muscle nature of the tumor. Glomus tumors arising in visceral organs are rare, and those arising in kidney are exceedingly rare.