Glomangiomyoma (glomus tumour) of the pancreas: a case report

Glomus tumours occur in many sites. We report the first case of a glomangiomyoma in the pancreas of a 17-year-old girl. The tumour was 5cm in diameter and consisted of rounded glomus cells, blood vessels and spindled smooth muscle cells. The glomus and smooth muscle tumour cells showed moderate diffuse cytoplasmic staining with vimentin, muscle-specific actin and smooth muscle actin. There was weak focal positivity for desmin in the spindle cell component only. The patient is alive and well with no evidence of recurrence 24 months after surgery.     

Thick (myosin) filaments in a glomus tumor

An otherwise classic digital glomus tumor is presented with the unusual ultrastructural finding of cytoplasmic thick (myosin) filaments together with thin (actin) filaments in many of the cells. In places, sarcomere-like orientation was seen. It is little appreciated among diagnostic pathologists, but is well-established, that thick (myosin) filaments occur in smooth muscle type cells. They are present in vivo and can be demonstrated ultrastructurally if rather stringent preparative conditions are met. Whether or not the contractile process in smooth muscle is analogous to skeletal muscle is a debated issue. In the context of diagnostic electron microscopy, it is stressed that thick filaments are not, as often stated, pathognomonic of skeletal muscle neoplasms, and may potentially be found in smooth muscle neoplasms and neoplasms of related cell type (glomus tumors, hemangiopericytomas, tumors of myofibroblasts, etc.).     

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.     

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.     

Malignant glomus tumor of kidney: the first reported case and review of literature

Glomus tumors are mesenchymal neoplasms and are rarely seen in visceral organs like the kidney. Our patient presented with a metastatic, malignant, and highly aggressive glomus tumor in the kidney. In our extensive literature review, we did not come across even a single case of malignant glomus tumor arising in the kidney. We report the clinical presentation, radiologic, and pathological features of our case. Immunohistochemical findings that distinguish our case from other reported cases of glomus tumors arising from the kidney have been discussed. We have also reviewed the criteria for malignancy and other reported malignant glomus tumors.     

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.     

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.     

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.     

Case report: glomus tumor of the colon

Glomus tumors are tumors of pericytic origin and are usually found in the distal extremities. Glomus tumors have rarely been reported in viscera. The authors report a glomus tumor of the colon that caused rectal bleeding in a 40-yr-old man and was biopsied and excised endoscopically. The histology and immunohistochemical profile of the tumor are described and the literature on visceral glomus tumors is reviewed.     

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.     

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.