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[Tracheal tumor--a case review].

Tracheal tumors make a histologically variant group of tumors. Our case review describes a case of a forty-five year-old female patient with an endoscopically and CT- diagnosed tracheal tumor. The patient was hospitalized at the IInd Surgical Clinic of the UPJS LF, the Faculty Hospital of L. Pasteur in Kosice, with symptoms of dyspnoea and stridor, in the orthopnoic position. Following the neccessary pre- operative procedures, her trachea was resected, followed by end-to-end anastomosis. The histological examination of the resected tissue revealed an inflammatory myofibroblastic tumor. The postoperative course was positive with no complications.

Female↗

Granular-cell tumours of the skin do not express carcino-embryonic antigen.

Granular-cell tumour (GCT) of the skin is an uncommon tumour of disputed histogenesis, that has been subjected to several immunohistochemical studies. The controversy existing in the literature concerning the expression of carcinoembryonic antigen (CEA) by GCT prompted us to study a series of 17 cases of cutaneous GCT by using an avidin-biotin-immunoperoxidase technique on routinely-processed tissue sections. No CEA activity was detected in any of the tumours screened. The reasons for this controversy are discussed.

Carcinoembryonic Antigen↗

Amianthoid myofibroblastoma of the soft tissues.

Myofibroblastoma (MF) is an uncommon, usually benign, mesenchymal tumor infrequently described in soft tissues. We report here on the clinicopathologic findings of a soft tissue MF (STMF) presenting in the neck of a 90-year-old man as a slowly growing and non-painful nodule, 4 cm in greatest diameter. Histology revealed a circumscribed lesion constituted of monomorphous bipolar spindle cells arranged in swirling fascicles with intervening broad bands of hyalinized collagen and well formed "amianthoid" fibers. Immunohistochemistry showed the spindle cells to be immunoreactive for vimentin, smooth muscle actin and muscle specific actin and, focally, for desmin; immunostaining for cytokeratin, epithelial membrane antigen, S-100 protein, factor VIII-related antigen, and CD34 was negative. Based on the present case and on those previously reported in the literature, STMF is characterized by: 1) exclusive incidence in the male sex; 2) variable immunoreactivity of the neoplastic cells for desmin, probably reflecting an origin from a peculiar subset of myofibroblasts, or, alternatively, a further myoid differentiation; 3) variable abundance of (hyalinized) collagen; 4) presence of amianthoid fibers. The combination of desmin immunoreactivity, frequently observed in MF of the breast, and amianthoid fibers, the main feature of MF of the lymph nodes, has never been observed in soft tissue MF. It is important to recognize STMF as a specific clinicopathologic entity to avoid confusion with other types of spindle cell proliferation and to differentiate it from other types of myofibromatosis.

Aged↗

[Present status of granular cell tumors. Apropos of 8 new cases with tracheobronchial localization].

The authors report 8 new cases of granular cell tumour of Abrikossoff's tumour located in the trachea or bronchus. After briefly recalling the generally accepted features of this disease, they report several new aspects: the obviously under-estimated frequency, the absence of progression, requiring therapeutic abstention and regular follow-up, the epidemiological problems posed by the association with chronic bronchitis or the coexistence with a malignant bronchial tumour, the uncertainties which still surround the histogenesis, which is partly mesenchymal and partly nervous tissue, more particularly Schwann cells, which can be supported by ultrastructural arguments.

Adult↗

Myofibroblastoma of the male breast: a diagnostic problem on fine-needle aspiration cytology.

Myofibroblastoma is an uncommon neoplasm of the male breast. Herein, we describe the cytologic features seen in the fine-needle aspirate of a 45-year-old man. The smears were cellular with intimate association of tumor cells with extracellular matrix material. The cells were spindle to polygonal and were without significant atypia. Numerous mast cells were observed. Nuclear grooving was present only occasionally, although this was conspicuous histologically. The presence of hyaline bands in between tumor cells, another interesting feature, was appreciated retrospectively. This neoplasm was initially misinterpreted as a malignant soft tissue tumor. Awareness of the cytologic features coupled with mammography should prevent a misdiagnosis of this tumor.

Biopsy, Needle↗

Soft tissue myofibroblastomas.

Five well-circumscribed solitary soft tissue tumors composed of myofibroblasts are described and termed myofibroblastomas. By light microscopy these lesions are characterized by short, intersecting, or crisscrossing fascicles of spindle cells, sometimes associated with foci of necrosis and/or mitotic activity with less than three mitoses per 10 high power fields. Myofibroblastomas show well-defined myofibroblastic differentiation ultrastructurally with peripheral myofilaments and vimentin, actin, and desmin immunocytochemistry positivity. The five tumors described occurred in patients of various age groups, including one congenital, and in a variety of soft tissue locations. It is important to recognize this benign soft tissue neoplasm to avoid confusion with other soft tissue tumors and to separate this lesion from other myofibromatosis. This study elucidates the spectrum of light microscopic, ultrastructural, and immunocytochemistry findings of soft tissue myofibroblastomas and establishes this soft tissue tumor as a specific clinico-pathologic entity.

Actins↗

An extraluminal leiomyosarcoma of the iliac vein without thrombosis--a case report.

Vascular leiomyosarcoma (LMS)is a rare malignant tumor arising from the muscle cells of the media of the vessels. Vascular LMS is often diagnosed as a result of the clinical manifestations of impaired venous flow such as edema and phlebothrombosis. The authors present a case of an LMS in a 72-year-old woman. Physical examination revealed a round mass deep in the left inguinal region close to the inguinal vessels, fixed and not pulsating. There was no sign of left lower-limb edema nor of articular impairment of the hip. Inguinal and distal pulses were normal. Results of laboratory analysis, including values for the oncologic markers, were normal. An ultrasound scan of the left inguinal fossa showed a 50-by-30 mm mass of mixed aspect that adhered to the left common iliac artery. An echo color Doppler showed conservative arterial flow. Thus, a thrombotic aneurysm of the left iliac artery was diagnosed. Chest x-ray showed no pathological findings. An abdominal computerized tomograph (CT) scan confirmed the location of the neoplasm and revealed a narrowing of the left iliac vein compressed behind the mass. The finding was interpreted as a colliquative lymph node. The patient underwent explorative laparotomy with midline incision. The finding was a neoplastic bilobed mass compressing and infiltrating the left iliac vein with no cleavage surface between the mass itself and the venous wall. An intraoperative frozen section revealed a necrotic mass with a cortex of ambiguous interpretation. Surgical procedure was then finished with no further venous resection. Histologic examination revealed the presence of leiomyosarcomatous tissue with nuclear pleomorphisms inside necrotic material limited by a thin fibrotic capsule. According to Coindre's classification the tumor was a G3. After six months the patient is fit and a CT scan showed no evidence of recurring disease.

Aged↗

Intermediate filaments in smooth muscle tumours.

Antisera to the intermediate filaments vimentin and desmin react with fixed paraffin embedded tissue. Benign uterine myomas contain both classes of filaments. Gastrointestinal "smooth muscle tumours" however often lack desmin even when they appear histologically benign. In the sarcomas examined vimentin was the only class of intermediate filament present. The diagnostic and histogenetic implications of these findings are discussed.

Adult↗

[Inflammatory myofibroblastic tumour (so-called pseudotumour) of the hepatobiliary system].

The purpose of this report is to describe the ultrasonographic features of hepatic inflammatory myofibroblastic tumour (IMT). This tumour presented as an area of periportal soft-tissue infiltration. Because periportal infiltration is a common feature in both IMT and other malignant tumours of the hepatic portal, histological examination should be considered before final diagnosis and treatment.

Gallbladder Neoplasms↗

Aggressive angiomyxoma: reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases.

Aggressive angiomyxoma is a distinctive soft tissue tumor associated with a high risk of local recurrence but lacks metastatic potential. This tumour occurs nearly exclusively in the soft tissues of the pelvis and perineum of adult women. The line of differentiation is not firmly established, but a fibroblastic/myofibroblastic origin has been proposed. We report 16 new cases of aggressive angiomyxoma of the pelvic soft tissue in women. In all cases bundles of cells, most often adjacent to vessels, with histological features of smooth muscle cells were identified. In 11 of 14 cases the myoid bundles were immunoreactive for desmin; they were also positive for smooth muscle actin in 10 of 11 cases. In 13 of 14 cases lesional stromal cells showed immunoreactivity for desmin. Three cases showed areas with histological features similar to those of angiomyofibroblastoma of the vulva, thus representing previously undescribed morphological overlap between these two entities. We conclude that aggressive angiomyxoma and angiomyofibroblastoma are related neoplasms in a spectrum of tumours showing myofibroblastic origin. Furthermore, the demonstration of immunoreactivity for desmin in aggressive angiomyxomas implies that this antibody is not helpful in discriminating between these two tumours, and the principal means of distinction remains histomorphological analysis.

Adult↗

Mediastinal masses in children.

A variety of mediastinal masses may present in children. Imaging is generally confined to chest radiograph and CAT scan of the chest, although other studies may prove helpful depending upon the circumstances. MRI is particularly helpful for neurogenic tumors in the posterior mediastinum. The most common tumor in the anterior mediastinum is lymphoma with germ cell tumors second. In general, lymph nodes in areas outside the mediastinum provide access for tissue diagnosis when lymphoma is present. Germ cell tumors are generally benign, but surgical excision is indicated to rule out malignant elements and to treat symptoms which are often present through compression of nearby structures. Neurogenic tumors are the most common lesions in the posterior mediastinum.

Bronchogenic Cyst↗

Granular cell myoblastoma of the vulva in a 6-year-old girl.

Granular cell myoblastomas are rare neoplasms thought to arise in neural tissue resembling Schwann cells. About 7% of these tumors are located on the vulva. This is the second reported case in a prepubertal girl with both cases having been found in 6 year olds. None of the vulvar lesions have been malignant. The treatment is wide surgical excision and observation for recurrence. Extragenital sites should be evaluated since multiple tumors are found in a number of patients.

Child↗

Inflammatory myoglandular polyps of the colon and rectum. A clinicopathological study of 32 pedunculated polyps, distinct from other types of polyps.

Hitherto unclassified colorectal polyps were identified in 32 patients (23 men and 9 women; mean age, 53 years). The only symptom, which was observed in less than half the patients, was passage of blood or occult blood. Endoscopic examination revealed solitary pedunculated, red polyps with a smooth surface. These polyps were found in the left colon, especially in the sigmoid. Their characteristic features were inflammatory granulation tissue in the lamina propria mucosae, proliferation of smooth muscle, and hyperplastic glands with occasional cystic dilatation. The etiology of this type of polyp is unknown, but it could involve chronic trauma from the fecal stream and from peristalsis of the bowel. These polyps can be differentiated from juvenile polyps and inflammatory polyps by the presence of abundant smooth-muscle cells in the inflamed lamina propria mucosae. They also can be differentiated from Peutz-Jeghers polyps, which appear as hamartomatous structures with tree-like proliferation of muscularis mucosae covered by colonic mucosa without inflammatory granulation tissue. Their locations and macroscopic appearance distinguish these polyps from mucosal prolapse syndrome and polyps developed after colostomy. In addition, these new polyps differ from inflammatory cap polyps in that they lack a fibrin cap. We propose the name inflammatory myoglandular polyps for these polyps, which are distinct clinicopathologically from other types of colorectal polyps.

Adolescent↗

Pictorial review: magnetic resonance imaging of benign soft tissue masses of the hand and wrist.

Magnetic resonance imaging (MRI) is the imaging method of choice for evaluating the presence and extent of soft tissue masses. It is particularly useful for assessing masses in the wrist and hand, where benign lesions predominate. A specific diagnosis may be made, or strongly suspected, from the characteristic MRI features found in certain conditions like ganglion, haemangioma, arteriovenous malformation, giant cell tumour of the tendon sheath and lipoma.

Arteriovenous Malformations↗

Angiomyofibroblastoma of the vulva: a large pedunculated mass formation.

Angiomyofibroblastoma is a rare, usually small benign mesenchymal tumor that occurs in vulvar lesions of premenopausal women. A case of angiomyofibroblastoma that arose as a unique pedunculated and particularly large mass in the left vulva of a 48-year-old woman is presented herein. The patient had been aware of a gradually enlarged mass of 7 years duration without any other gynecological symptoms or signs. The maximum dimension of the tumor measured 11 cm. The resected tumor was well circumscribed with a bulging and glistening cut surface. Histological examination revealed an admixture of irregularly distributed hypercellular and hypocellular areas with spindled, plump spindled, or plasmacytoid stromal cells and abundant venular or capillary-sized vessels. Stromal cells characteristically cluster around delicate vessels within an edematous to collagenous matrix. In the present case, intralesional adipose tissue was present throughout the tumor. There was no significant nuclear atypia, and mitotic figures were very sparse. There was little stromal mucin throughout the tumor. Immunohistochemically, the stromal cells were characterized by strong reactivity for vimentin and CD34, with focal reactivity for desmin and alpha smooth muscle actin. Both estrogen and progesterone receptors were diffusely expressed in the stromal cells. These histological findings are consistent with angiomyofibroblastoma and support the hypothesis that angiomyofibroblastoma originates from perivascular stem cells with a capacity for myofibroblastic and fatty differentiation.

Female↗

Myofibroblastic tumours: an update.

The concept of the myofibroblast is relatively new, since this cell type was defined less than 30 years ago, and there is as yet no firm consensus as to how a myofibroblast should be defined either morphologically or phenotypically. Because its attributes place it midway between a fibroblast and a smooth muscle cell and because it appears capable of functional and phenotypic modulation, some authorities argue that there is no such thing as a truly myofibroblastic tumour. However, since most diagnostic tumour pathology is based on phenotypic features of the excised lesion, it is an unavoidable fact that a wide range of soft tissue tumours show convincing myofibroblastic features. Lesions in this category fall into four main groups--the family of reactive fasciitis-like lesions, a group of benign lesions most of which have been fairly recently recognized (e.g., mammary myofibrolastoma, intranodal myofibroblastoma, angiomyo-fibroblastoma and dermatomyofibroma), the locally aggressive fibromatoses (either superficial or deep) which share features of fibroblasts and myofibroblasts in varying degree and, finally, sarcomas showing myofibroblastic differentiation. These latter include low grade lesions such as so-called infantile fibrosarcoma, inflammatory myofibroblastic tumour and a distinctive form of low grade myofibroblastic sarcoma, as well as some of the high grade lesions formerly known as MFH. Based on the advances made in the past 20 years, it makes no sense to deny the existence of myofibroblastic tumours (especially in an era when histogenetic concepts have crumbled)--instead we need to work towards a reproducible and agreed definition of the myofibroblast.

Child↗

Rare thoracic mass lesion--myofibrobastoma.

Mesenchymal soft tissue masses are uncommon tumours of the chest. Myofibroblastoma is a recently described entity consisting of cells with origin from the myoepethelial cell, mostly seen as benign well-circumscribed neoplasms of the breast tissue. Though usually classified as a benign lesion, rarely it can be multifocal and prone to recurrence. We describe below the case of a 26-year-old female who presented with exertional dyspnoea and evidence of a massive mass lesion in the left hemithorax causing mediastinal displacement to the opposite side. On histological and immunophenotypic analysis, a diagnosis of the very rare thoracic myofibroblastoma was made. The incidence, salient features, pathological differential diagnoses and treatment are reviewed.

Adult↗