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[Various aspects of the morphology of granular cell tumors].

Histology and ultrastructure of human granular cell tumors (GCT) are studied. Apart from "granular" cells which are the main component of the tumor, GCT contain the cells of ordinary structure with signs of neurogenic differentiation, cell processus typical for the nervous tissue and peripheral nervous tissue tumors (these processus are in a close space connection with "granular" cells) as well as structurally transitional cells. This implies that "granular" cells are likely to originate from the ordinary lemmocytes as a result of disturbance and hypertrophy of intracellular processus. Preoperative diagnosis of this rare peculiar neurogenic tumor is difficult and the diagnosis is possible only after the operative material examination. If other signs of malignancy are lacking, infiltrative growth typical for these tumors allows one to consider them locally-destructive neoplasms.

Adolescent↗

Inflammatory myofibroblastic tumor presenting as a pancreatic mass: a case report and review of the literature.

CONTEXT: Inflammatory myofibroblastic tumor is a distinctive lesion of unknown etiology. It has generally been considered a rare benign pseudosarcomatous lesion of admixed inflammatory infiltrates with myofibroblastic spindle cells. Although original case descriptions focused on the pulmonary system, it is now recognized that virtually any anatomic location can be involved. However, an inflammatory myofibroblastic tumor located in the pancreas is rare. CASE REPORT: We report a case of an asymptomatic 70-year-old Caucasian man with a 3.8 cm inflammatory myofibroblastic tumor located in the tail of the pancreas which was discovered incidentally on a computed tomography scan of the abdomen. Endoscopic ultrasonography with fine needle aspiration was negative for malignancy. However, because of worrisome radiographic features, a distal pancreatectomy with splenectomy was performed. The pathology revealed an inflammatory myofibroblastic tumor with focal extension into the peripancreatic soft tissues, but with negative surgical margins. The patient has been followed for 10 months without evidence of recurrence. CONCLUSIONS: To date, there have been only 25 cases of inflammatory myofibroblastic tumor located in the pancreas reported in the English language scientific literature. Even with multimodal pre-surgical investigation, it can be extremely difficult to differentiate inflammatory myofibroblastic tumor from pancreatic malignancies. Most cases require surgical exploration and complete resection to obtain an accurate diagnosis. A review of published case reports is also presented.

Aged↗

Granular cell myoblastoma: rare localization in the trachea. Report of a case and review of the literature.

Granular cell myoblastoma is an uncommon tumor in the respiratory tract. It usually occurs in the tongue, skin, breast, or subcutaneous tissue. When it occurs in the respiratory tract, it is usually located in the bronchus or larynx. Primary tracheal location is rare with only nine such cases reported in the literature. This report describes a 26-year-old woman with granular cell myoblastoma of the trachea. She presented with a four-year history of bronchial asthma. The tumor was surgically excised by tracheal resection and reconstruction. The patient has remained well and free of obstructive airway symptoms, without recurrence of the tumor for more than one year.

Adult↗

Mammary-type myofibroblastoma of soft tissue: a tumor closely related to spindle cell lipoma.

Mammary myofibroblastoma is a benign breast tumor, with a reported predilection for older men. It is composed of fascicles of spindle cells having features of myofibroblasts, with intervening hyalinized collagenous stroma and a variably prominent component of adipose tissue. The spindle cells characteristically express both CD34 and desmin. Herein, we report the clinicopathologic features of nine tumors that were morphologically and immunohistochemically identical to myofibroblastoma of breast; however, they arose in subcutaneous soft tissue at extramammary sites. The study group comprised seven men and two women with an age range of 35-67 years (median 53 years). Lesions presented as either a slowly growing painless mass or were incidental findings at the time of surgery. The site distribution was as follows: inguinal/groin area (five cases) and one case each in posterior vaginal wall, buttock, anterior abdominal wall, and mid-back. Tumor size ranged from 2 to 13 cm (median 6 cm), and all lesions were well circumscribed. Eight tumors had a component of adipose tissue (ranging from 10% to 60%), within which some variation in adipocyte size was often seen. One case showed epithelioid cytomorphology and three cases showed rare atypical or multinucleated cells. Focal myxoid stromal change was seen in four cases. Tumor cells were positive for desmin (9 of 9 cases), CD34 (8 of 9 cases), and occasionally positive for smooth muscle actin (3 of 9 cases). Lesions were marginally excised with no recurrences to date, although follow-up is very limited. Lesions with morphologic and immunophenotypic features similar to myofibroblastoma of breast can arise at extramammary sites, with an apparent predilection for the inguinal area of older men. Both mammary and extramammary lesions show morphologic overlap with spindle cell lipoma and are likely closely related.

Adult↗

The role of electron microscopy in gynecological pathology.

Electron microscopy, as a diagnostic method, has been available to pathologists for about half a century. Its use in studying normal and abnormal gynecological tissues has been applied during the second half of that period, and many works on specific female genital topics have been published. Several of those subjects are worthy of citing in a review of the present type. Clear cell carcinoma has been revealed to be a mullerian, rather than a wolffian, derivative. Small cell carcinoma of the ovary with hypercalcemia is comprised of cells shown ultrastructurally to be epithelial, but unlike surface epithelial cells, germ cells, sex-cord cells, or neuroendocrine cells. Further electron microscopic studies provided evidence that these small cell tumors are not adult diffuse granulosa cell tumors, endometrioid stromal tumors, primitive neuroectodermal tumors, or numerous other primary and metastatic small cell tumors. Electron microscopy has also been useful in determining that not all signet-ring cell tumors of the ovary are stromal, and that there are multiple types of signet-ring (vacuolated) cells in ovarian tumors. Smooth muscle tumors are well known to have multiple light microscopic phenotypes, and electron microscopy has proven to be diagnostic in many of these cases, especially in epithelioid smooth muscle tumors. A number of other gynecological neoplasms that have been better defined by electron microscopic studies are described. Embryology and histogenesis are other areas of study in which electron microscopy has been a major contributor of new information at the subcellular level. Electron microscopy, solely or in harmony with clinical information, light microscopy, and immunohistochemistry, has been and is a valuable tool for the pathologist in the study of histogenesis and accurate diagnosis of gynecological lesions.

Adenocarcinoma, Clear Cell↗

Granular cell tumor of the biliary tree. A report of two cases and a review of the literature.

Granular cell tumors are relatively uncommon soft tissue tumors usually presenting in the skin and subcutaneous tissues or tongue, although many sites have been described. Two cases arising in the extrahepatic biliary tree are described, and the previously reported cases of this rare presentation are reviewed. These tumors may mimic sclerosing cholangitis and cholangiocarcinoma clinically, and occasionally histologically, in this relatively young group of patients. The histogenesis appears to be related to Schwann cells as in granular cell tumors of other sites, evidenced by histologic, immunohistochemical, and electron microscopic findings. Granular cell tumors, albeit rare, should be considered in the differential diagnosis of biliary tract disease in young patients, particularly black women, and are curable by surgical excision.

Adult↗

Intra-abdominal inflammatory myofibroblastic pseudotumor: case report and review of the literature.

Inflammatory myofibroblastic pseudotumors (IPM) are very rare tumor characterized by unpredictable clinical behaviour. They arise in soft tissues of almost every organ and the most common site is the lung. Over 200 cases of inflammatory myofibroblastic pseudotumor of the lung have been described in literature. Intra-abdominal IMP are very rare. We describe a case of intra-abdominal IMP in a boy of 15 years old who presented symptoms and signs of acute appendicitis. Exploratory laparotomy revealed a mass in the peritoneal cavity. The mass was removed. The histologic examination showed that it was an IMP. Surgical treatment was the only therapy. Six months after the surgical operation the patient has no sign of illness.

Adolescent↗

[Immunohistochemical differentiation of leiomyocellular tumors and tumors with myogenic differentiation].

The expression of alpha smooth muscle actin, muscle specific actin, desmin, h-caldesmon, and calponin was studied immunohistochemically in the following soft tissue and bone tumours and tumour-like lesions: muscle fibromatosis, inflammatory pseudotumours, chondroblastoma, enchondroma, chondrosarcoma, fibrous dysplasia, ossifying myositis, osteoblastoma, convential osteosarcoma, leiomyoma and leiomyosarcoma. Tumours and tumour-like lesions with myofibroblastic cells, osteoblasts and chondroblasts frequently exhibited intensive immunoreactivity for the muscle markers, and therefore, some of them may occasionally be confused with leiomyoma and leiomyosarcoma. Calponin does not help to differentiate various mesenchymal tumours expressing muscle markers, because it also stains intensively myofibroblasts, osteoblasts and chondroblasts. We confirmed that h-caldesmon was expressed intensely in leiomyomas and leiomyosarcomas, and never in the other tumours examined, with the exception of three chondroblastomas. The results have shown that h-caldesmon is a rather specific and sensitive marker for smooth muscle tumours, but it can also stain some actin positive myochondroblasts. It is possible that the positivity of h-caldesmon in some chondroblastomas is due to their complete myogenic transdifferentiation, and so we use the term myochondroblasts and myochondrocytes for designation of such S-100 protein, actin, and h-caldesmon positive cells.

Actins↗

Angiomyofibroblastoma of the vulva: a mitotically active variant?

A case of angiomyofibroblastoma in a 48-year-old woman is reported. The tumor occurred as a left vulval mass and was treated by simple excision. It was located in the subcutaneous tissue of the left vulva and was well circumscribed, measuring 2.8 x 2.7 x 2.5 cm. Microscopically, the tumor was composed of hypocellular and cellular areas with well-developed small vessels. Spindle or polygonal cells were arranged with perivascular accentuation in an edematous or fibrocollagenous background. Some spindle-shaped or polygonal stromal cells were also arranged in epithelioid nests. In some areas, mitoses were frequent (maximum 3/10 high-power field). Immunohistochemically, the stromal cells were positive for vimentin and desmin, but negative for alpha-smooth muscle actin, S-100, neurofilament, estrogen receptor, progesterone receptor, CD31 and CD34. The average labeling index of Ki-67 in stromal cells was 3.1%. Ultrastructural analysis demonstrated that the stromal cells adhered with primitive junctions and contained intermediate filaments with no focal density in the cytoplasm. These findings were consistent with angiomyofibroblastoma, although previously reported cases did not show so many mitoses. Therefore, this case was suggested to be a mitotically active variant.

Angiofibroma↗

The myofibroblast: a study of normal, reactive and neoplastic tissues, with an emphasis on ultrastructure. part 2 - tumours and tumour-like lesions.

This paper describes the ultrastructure of the commoner myofibroblastic tumours and tumour-like lesions. The objective is to complement mainstream pathology texts, which have concentrated on the clinical and light microscopy features of these lesions and which have arguably but understandably somewhat neglected electron microscopy as an ancillary diagnostic tool and a technique for investigating tumour cell biology. Ultrastructural features are described of nodular fasciitis, the myofibromatoses (including Dupuytren's disease), inflammatory myofibroblastic tumour, post-operative spindle cell nodule, fibroma of tendon sheath, fibrous pseudotumour, benign fibrous histiocytoma, atypical fibroxanthoma, dermatofibrosarcoma protuberans, myofibrosarcoma (myofibroblastic sarcoma), malignant fibrous histiocytoma (pleomorphic myofibrosarcoma), epithelioid sarcoma and spindle-cell carcinoma. Fibrosarcoma and leiomyosarcoma are illustrated for comparison. The fibronexus is emphasised as an important marker for the most confident diagnosis of myofibrosarcoma. Some pathologists accept a light microscope definition, which includes alpha-smooth-muscle actin positivity, h-caldesmon negativity and, in some cases, desmin positivity. Caution in the interpretation of desmin staining in a possible myofibroblastic lesion is urged, since, in combination with an ultrastructurally identified lamina, it more probably suggests true smooth-muscle differentiation. Myofibroblastoma and angiomyofibroblastoma are examples of tumours argued on the basis of ultrastructural findings (sometimes in combination with desmin staining) to be primitively differentiated smooth-muscle cell rather than myofibroblastic proliferations.

Adolescent↗

Granular cell tumors.

A lesion of unknown etiology and histogenesis, the granular cell tumor usually arises in the skin or soft tissue. It has been reported, however, in other sites and can be multifocal. The authors have seen 31 such tumors in 26 patients in their institutions since 1970. Most (21) patients were females, and 12 patients were black. The average age was 41.8 years, excluding two newborns with a congenital granular cell myoblastoma of the gingiva. The most common site of occurrence was the skin; seven tumors originated from the trunk and five from the extremities. Four lesions were found in the breast, three on the vulva, two in the axilla, two in the gum, two in the buccal cavity, two in the esophagus, and one each in the stomach, gluteus muscle, eyelid, and bronchus. Two patients had multiple synchronous lesions. These were bilateral hand lesions in one patient, and lesions of the breast and axilla in the other. A third patient had three separate lesions which arose over the course of 5 years, involving the bronchus, the gluteus muscle, and the buccal mucosa. An additional esophageal lesion was an incidental finding 3 years after excision of a granular cell tumor of the breast. All of the tumors were removed with local, simple excision, except for the 2-cm lesion in the stomach for which a wedge resection of the fundus was necessary and the bronchial lesion for which a wedge resection of the left upper lobe of the lung was performed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Granular cell myoblastoma of the orbit. Report of a case.

A light and electron microscopical study of a case of granular cell myoblastoma of the orbit is described. Its possible histogenesis is discussed and, from the study of this case and of the literature, it is believed that the most likely histogenesis is from a primitive mesenchymal cell resembling a fibroblast.

Histocytochemistry↗

Granular cell tumor (myoblastoma) of the orbit.

A 43-year-old woman had a three-month history of increasing proptosis and visual loss secondary to an inferior orbital mass. Surgical exploration revealed an encapsulated mass that was excised with good visual recovery. Light microscopy as well as electron microscopy confirmed the diagnosis of granular cell myoblastoma. The tumor's location within the orbit was unusual and its histologic origin was uncertain.

Adult↗