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[Glomus tumours: morbid-anatomical analysis of 58 cases, especially infiltrated forms (author's transl)].

Analysis of 58 glomus tumours revealed a higher incidence among males. The upper limbs were the most frequent site. The tumour is usually small, lies in subcutaneous tissue, occasionally subungual, and is often very painful. There may be more than one tumour. Most of the tumours are encapsulated and of the angiomatous type. Cases with predominantly vessels of type I (after Masson) correspond to the so-called angiomyoma of Schumacher. This form of the tumour is frequently located in the lower limbs. In three younger patients infiltrating soft-tissue tumours were found in the lower limbs. In all three histological examination revealed non-encapsulated, angiomatous glomus tumour, type angiomyoma. There were no signs of malignancy and no metastases. Even though glomus tumours are classified among the benign tumours, there are non-encapsulated, angiomatous forms with a tendency to local infiltration and local recurrences. Clinically they are thus semi-malignant.

Adolescent↗

Leiomyoma of the oral cavity: a light microscopic and immunohistochemical study with review of the literature from 1884 to 1992.

Leiomyoma is the most common benign neoplasm in the uterus and stomach but is rare in the oral cavity. There were only 5 oral cases in a series of 7748 leiomyomas of all sites. Benign smooth muscle neoplasms are classified into leiomyoma (solid leiomyoma), angiomyoma (vascular leiomyoma) and epithelioid leiomyoma (leiomyoblastoma). 6 cases diagnosed as leiomyoma were retrieved from the files of two oral biopsy services over the past 25 years. A light microscopic study including trichrome and phosphotungstic acid haematoxylin (PTAH) stains, and an immunohistochemical study with the following markers: desmin, muscle specific actin, myoglobin, vimentin, S-100 protein, neuron-specific enolase, factor VIII and Ulex europeus were done with suitable controls. The haematoxylin and eosin and Masson's trichrome stains supported a diagnosis of leiomyoma in all 6 cases but PTAH was positive in only 3 of them. The immunohistochemical study confirmed the diagnosis of leiomyoma in 3 cases. The other 3 were identified as granular cell tumour, myofibroma and neurofibroma, respectively. The review of the literature contributed the following data: mean age was 41 and median age 39 in 134/142 patients. A male sex prevalence 72/137 patients (54.0%) was noted. The lips were the most common site with 39 cases (27.46%) followed by the tongue 26 (18.30%), cheeks and palate 22 (15.49%), gingiva 12 (8.45%), and mandible 8 (5.63%). Prognosis of oral leiomyomas is excellent. Immunohistochemistry is a precise and reliable method for definitive diagnosis of oral leiomyoma.

Adolescent↗

[A case of pulmonary lymphangiomyomatosis diagnosed by biopsy of retroperitoneal tumor and treated with hormonal therapy].

A 34-year-old woman was admitted to our hospital because of exertional dyspnea and ascites. Chest roentgenogram showed hyperinflation of the lung and diffuse reticular shadows. Abdominal CT showed retroperitoneal tumors. Ascites was considered to be chylous. These findings strongly suggested the diagnosis of pulmonary lymphangiomyomatosis. Under lumbar anesthesia, laparotomy was performed, and the histological finding of the retroperitoneal tumor revealed angiomyoma. Bilateral oophorectomy was performed. Medroxyprogesterone at 15 mg/day was administered postoperatively. In advanced cases of this disease, open lung biopsy or even transbronchial lung biopsy is often avoided because of severe cardiopulmonary dysfunction. However, such cases associated with retroperitoneal tumor, laparotomy is quite useful not only in making the diagnosis but also in the treatment of this disease.

Adult↗

Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma).

A series of 127 surgical specimens of epithelioid leiomyomatous tumors (leiomyoblastomas) of the gastric wall from the files of the Armed Forces Institute of Pathology (AFIP) were studied as to biologic behavior, morphogenesis, and histologic features of value in distinguishing benign and malignant variants. These tumors affect middle-aged men primarily and usually present with upper gastrointestinal bleeding or peptic ulcer-like symptoms. They are composed of a mixture of round epithelioid and spindle cells, many of which have clear cytoplasm. The cells are ensheathed by delicate reticular fibers. The presence of a perithelial or glomoid pattern in some tumors suggests a possible relationship to angiomyoma, glomus tumors, and "pericytoma." The epithelioid leiomyoma, the benign form, often arises in the mid- and distal stomach, especially on the anterior wall. Microscopically, it is recognized by the presence of large epithelioid cells and infrequent mitotic figures. Of 103 epithelioid leiomyomas, only one metastasized and thus was biologically malignant. The epithelioid leiomyosarcoma often arises in the proximal stomach and also distally, especially on the posterior wall. Two histologic types of epithelioid leiomyosarcoma are distinguished from the benign epithelioid leiomyoma by the small size of the cells and occasional higher mitotic counts. One sarcoma variant is a small cell caricature of the leiomyoma. The other is more anaplastic, assoicated with a loss of reticular fibers surrounding the cells and an alveolar arrangement. Epithelioid leiomyosarcomas are the most common type of gastric sarcoma. They are aggressive neoplasms; 63% metastasized, usually within 2 years after diagnosis.

Adolescent↗

Distribution of collagen type IV in soft tissue tumors. An immunohistochemical study.

The distribution of collagen type IV, one of the major constituents of basement membrane, was studied immunohistologically in a series of 103 soft tissue tumors including those of peripheral nerve origin, smooth muscle origin, striated muscle origin, fibrous tissue origin, fibrohistiocytic origin, adipose tissue origin, synovial tissue origin, and blood vessel origin, paragangliomas, alveolar soft part sarcomas, granular cell tumors, and epithelioid sarcomas. Intensely positive staining for collagen type IV was observed in neurilemomas, neurofibromas, malignant schwannomas, and blood vessel tumors. Weakly to moderately positive staining was seen in leiomyomas, angiomyomas, and leiomyosarcomas. In contrast, synovial, fibroblastic and fibrohistiocytic tumors, benign or malignant, were negative. In paragangliomas, granular cell tumors, and alveolar soft part sarcomas, positive staining was evident surrounding nests or clusters of tumor cells. In all tumors, staining for collagen type IV clearly illustrated the vascular pattern.

Collagen↗

Oral leiomyomas.

Oral leiomyomas are considered to be rare neoplasms, but they may be encountered more frequently than generally believed. Three types of leiomyomas are commonly described: solid leiomyomas, angiomyomas, and epithelioid leiomyomas. Three cases of solid leiomyoma are presented, all of which occurred in the anterior mandibular mucobuccal fold. Leiomyomas can be easily confused with other spindle-cell tumors. The necessity of using special stains, especially Mallory's phosphotungstic acid hematoxylin, is discussed.

Adult↗

[Benign mesothelioma of the uterus. Clinical and histopathological aspects and evidence of its mesothelial origin. Apropos of 1 case].

Benign mesothelioma is a rare tumor nearly always find in relation to the genital tract. We report the case of a 47-year, old woman admit in the gynecologic department for metrorragia. The tumor was composed of irregular tubules between smooth muscle cells, lined by flattened or cuboidal cells without nuclear atypia or mitoses. The tumor cells express cytokeratins and vimentin. Histologically, differential diagnosis can be made with adenocarcinoma or vascular proliferation like lymphangioma, hemangioma or angiomyoma. Clinical outcome is always favourable without recurrence or malignant transformation.

Diagnosis, Differential↗

Chemical shift GRE MRI of renal angiomyolipoma.

The renal angiomyolipoma is a fairly common benign renal tumor composed of various proportions of smooth muscle, blood vessels, and fat. Because fat is present in virtually every case, the CT findings in angiomyolipomas are so distinctive that a histologic diagnosis may be suggested. In angiomyomas composed mainly of vascular tissue and muscle or in those in which recent hemorrhage has occurred, the majority of tumors may have soft tissue CT density values without apparent fatty component (1,2). In such cases, preoperative diagnosis is extremely difficult. In this case report, we detected a small amount of fatty tissue with a chemical shift GRE MRI technique, although CT failed to detect the fatty tissue.

Aged↗

Immunohistochemical staining of cutaneous tumours with G-81, a monoclonal antibody to dermcidin.

BACKGROUND: Recently, the novel antimicrobial peptide named dermcidin (DCD) was reported in human eccrine sweat glands. OBJECTIVES: We investigated the expression of DCD in a variety of cutaneous tumours in order to assess the usefulness of the monoclonal antibody (G-81), which recognizes a fragment of DCD. PATIENTS/METHODS: We studied the immunoreactivity of the G-81 antibody on 197 cutaneous tumours. RESULTS: A total of 13 of 26 cutaneous mixed tumours showed substantial immunoreactivity. In contrast all the following cases were completely unreactive: (i) epithelial tumours (seborrhoeic keratosis, squamous cell carcinoma, Bowen's disease, actinic keratosis, genital Paget's disease); (ii) follicular tumours (basal cell carcinoma, trichilemmoma, trichoepithelioma, trichoblastoma, keratoacanthoma, proliferating trichilemmal tumour, pilomatricoma); (iii) melanocytic tumours (malignant melanoma, naevus cell naevus, Spitz naevus, blue naevus); (iv) neural tumours (schwannoma, neurofibroma, Merkel cell neoplasm); (v) mesenchymal tumours (soft fibroma, dermatofibroma, dermatofibrosarcoma protuberans, vascular leiomyoma, leiomyosarcoma, lipoma, juvenile xanthogranuloma, angiomyoma); and (vi) other sweat gland tumours (poroid neoplasms, syringoma, cylindroma, clear cell hidradenoma, spiradenoma, syringoid eccrine carcinoma, mucinous carcinoma, apocrine cystadenoma, syringocystadenoma papilliferum, apocrine adenocarcinoma). Twenty-six cutaneous mixed tumours were considered from histopathological findings to be the apocrine type, but 13 of 26 mixed tumours contained some DCD-immunopositive cells that possibly differentiate into eccrine secretory glands. CONCLUSIONS: We found the expression of DCD in tubular structures of 50% of cutaneous mixed tumours with apocrine differentiation. These results suggest that a number of cutaneous mixed tumours show both eccrine and apocrine differentiation in the same neoplasm.

Antibodies, Monoclonal↗

[Monstrous venous hemangioma of the retroperitoneum: problems of diagnosis].

The venal haemangioma is defined as a benign tumor consisting of veinlike vessels. The preoperative diagnosis and its inherent problems are illustrated using a coincidentally diagnosed monstrous haemangioma tumor of the retroperitonial space in a twenty year old patient. With respect to our patient, X-ray, computer tomography and angiography all failed as diagnostic tools. Only the use of Doppler sonographic flow signals suggested the presence of a haemangioma. The morphology, prognosis and clinical significance of blood vessel tumors are multifaceted. The most important differential diagnoses to the venous haemangioma are the cavernous and the capilliary haemangioma. The venous haemangioma distinguishes itself through the presence of blood vessel walls. Haemangiomas are common benign tumors. In the presence of highly developed muscular components, there exists a transition to angiomyomas and to leiomyomas. Venal haemangiomas are extremely rare in the demonstrated localisation of the retroperitoneal space. Here they can grow to monstrous preportions whilst remaining undetected. Thus the patient is under the potential danger of bleeding to death through trivial injuries. The therapy of choice remains total surgical excision. In situations of unclear clinical results with respect to preoperative diagnosis especially amongst young adults, one should at least consider the possibility of a haemangioma as the root of the problem.

Adult↗

Cutaneous angiomyolipoma. A light-microscopic, immunohistochemical, and electron-microscopic study.

We report a case of cutaneous angiomyolipoma found on the helix of a 67-year-old man. The lesion was studied by routine light microscopy, special stains, immunohistochemical methods, and electron microscopy. Histologic examination showed a well-circumscribed nodule in the dermis composed of an intimate mixture of blood vessels, smooth muscle, and mature fat. These components were confirmed by special stains, immunohistochemistry, and electron microscopy. We concluded that the unique features of this lesion distinguish it from other lesions such as angiomyoma, angiolipoma, and other mixed mesenchymal tumors. This report demonstrates that the features considered diagnostic of angiomyolipoma can occur in extrarenal sites and, therefore, this diagnosis cannot be excluded on the basis of site alone.

Aged↗

Tenosynovial fibroma.

Tenosynovial fibroma or angiofibroma of tendon sheath is a distinctive clinicopathological entity occurring mainly in young and middle-aged men and affecting dominantly the distal extremities. Fingers, hands and wrists are the site of 80 per cent of cases. Palmar aspects of hands and plantar aspects of foot are preferentially involved. The trunk and neck are seldom affected. They are generally small, firm, sometimes painful lesions attached to tendon sheath and/or tendon. The lesion must be distinguished from a variety of benign lesions including nodular fasciitis, angiomyoma and giant-cell tumour of tendon sheath. It can also be confused with sarcoma, including synovial sarcoma, because of the presence in it of characteristic slit-like spaces which have been identified as vascular rather than synovial spaces by virtue of the Factor VIII content of their lining cells.

Adult↗

Vascular leiomyoma of the superior turbinate: first reported case.

BACKGROUND: Vascular leiomyoma is an uncommon smooth muscle tumor rarely found in the head and neck area. We report the first case arising for the superior turbinate of the nasal cavity. METHODS: A case presentation, treatment, and review of the literature are discussed. RESULTS: Twenty-one months after embolization and surgical resection, the patient is doing well, without evidence or recurrence. CONCLUSIONS: Vascular leiomyoma is a rare benign tumor of the nasal cavity, and surgical excision yields high cure rates.

Adult↗

Primary intracranial pleomorphic angioleiomyoma--a new morphologic variant. An immunohistochemical and electron microscopic study.

BACKGROUND: Angioleiomyomas usually are benign subcutaneous neoplasms that occur most often in extremities of middle-aged individuals. Very few cases have been described in other locations; none along the neuroaxis. An intracranial example of angioleiomyoma displaying unusual morphologic features not seen in the typical peripheral variants of this tumor is described. METHODS: The tumor was studied with conventional histology, immunohistochemistry with morphometric calculation of proliferation index, immunoelectron microscopy, and DNA flow cytometry. RESULTS: The tumor was composed of large epithelioid and pleomorphic cells filled with intermediate filaments positive for desmin and vimentin. Scattered cells also expressed myosin and muscle-specific actin. Smooth muscle cell differentiation was confirmed by ultrastructural demonstration of subplasmalemmal dense bodies, attachment plaques, and discontinuous basal lamina. The proliferation index with proliferating cell nuclear antigen (PCNA) monoclonal antibody was 75.78%, whereas it was only 4.22% with Ki67 monoclonal antibodies adopted to paraffin material (MIB-1). CONCLUSION: The tumor represents a unique morphologic variant of a pleomorphic angioleiomyoma. Cellular pleomorphism and a strong reaction for PCNA in numerous cells suggested that the lesion was malignant. However, the absence of mitotic figures, a small number of Ki-67-positive cells, a diploic DNA pattern, and a low proliferation index in flow cytometry all supported the concept that this neoplasm represented an unusual histologic variant of benign angiogenic leiomyoma. Encapsulation and demarcation of the surgical specimen and the survival of the patient for more than 4 years without recurrence after resection support this interpretation.

Adult↗

Cytologic features of angioleiomyoma: cytologic-histologic study of 10 cases.

Fine-needle aspiration (FNA) features in 10 angioleiomyomas (ALM) were evaluated and correlated to histological features and clinical data. The tumors developed in the skin or subcutaneous tissue of the lower extremities (eight), the shoulder (one), and the lower arm (one). Eight tumors were painful or tender. Cytological specimens showed moderate or sparse cellularity. The most frequent findings were uniform spindle cells, seen in nine smears. In eight of these, the spindle cells were admixed with smooth muscle cells and fragments of collagenous tissue in varying proportions. The occurrence of macrophages and fat cells was less specific, being present in three of nine smears. One aspirate contained some "ganglion-like" cells and a multinucleated giant cell. Smears from ALM are not sufficiently characteristic to permit a histotype diagnosis, but together with typical clinical data (painful or tender nodule) may help to exclude other skin or subcutaneous lesions.

Adult↗

Subcutaneous angioleiomyomas: gray-scale and color Doppler sonographic appearances.

PURPOSE: To describe the gray-scale Doppler and sonographic features of a series of subcutaneous angioleiomyomas. METHODS: The sonographic appearances of 10 pathologically proven angioleiomyomas were retrospectively reviewed; 4 in women and 6 in men, with an age range from 33 to 77 years. We evaluated size, shape, echo pattern, margins, location, relationships with adjacent structures, and vascularity. Examinations were performed using a multifrequency linear array transducer (9-11 MHz) connected to a Logiq 500 scanner (GE, Milwaukee, Wl). RESULTS: All tumors were subcutaneous and located in the extremities (7 in the lower extremities, 3 in the upper extremities). The sizes ranged from 0.6 to 6.4 cm, with an average size of 2 cm. All of the lesions were hypoechoic with well-defined margins, and 9 were oval. Intratumoral calcifications were observed in two patients. Vascularity was easily detected in all of them, and 4 tumors had a clear vascular pedicle. The spectral Doppler analysis performed in 5 cases, revealing a low-resistance arterial waveform in 4 patients. CONCLUSIONS: Although angioleiomyomas are uncommon soft tissue tumors, the presence of a well-defined, hypoechoic, vascular subcutaneous tumor in the extremities should raise the possibility of such a diagnosis.

Adult↗

Angiomyofibroblastoma of the vulva.

Angiomyofibroblastoma is a rare, myxoid tumor of the vulva. To date only 12 cases have been reported in the world literature. Patients are usually premenopausal and present with a vulval mass initially diagnosed as a Bartholin's cyst. The lesions are well circumscribed and range from 0.5 to 12 cm in size. Microscopically the tumors are characterized by high cellularity, numerous blood vessels, and plump stromal cells. Treatment is by surgical excision. There are currently no published reports of local recurrence or metastatic disease. Angiomyofibroblastoma should be differentiated from other neoplasms of the vulva where radical surgical treatment is indicated. A Case Report of angiomyofibroblastoma of the periclitoral region diagnosed in a postmenopausal woman is presented.

Aged↗