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The desmoid tumor is a rare neoplasm which, because of its histopathologic appearance, has been traditionally considered to be benign. Despite its benign microscopic features, it has an aggressive local behavior and, if not excised adequately, has a tendency to recur locally and invade neighboring structures with significant potential for morbidity, deformity, or even death. Two cases of recurrent extraabdominal desmoid tumors are presented not only because they are highly representative of this disease, but also because they emphasize the need for aggressive surgical treatment. Also, they are unusual and challenging cases from a reconstructive standpoint. Based on this experience and on the most recent literature, we believe that this tumor, regardless of its microscopic features, should be addressed and treated as a malignancy.
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Surgery for benign neoplasm extending into the orbital roof requires immediate reconstruction to avoid complications, which include transmission of the cerebral pulse to the globe, bulbar dystopia, diplopia, and fibrosis of the oculomotor muscles. Many alloplastic materials have been employed for such reconstruction, but currently most authors agree that autologous bone graft is the best option. Using calvarial bone in adults and split ribs in children, we have operated on eight patients for fibrous dysplasia (five cases), neurofibroma (two cases), or meningioma (one case). After a median follow-up period of two years and six months, good morphology of the orbit was maintained with no ocular symptoms.
BACKGROUND: The aim of this study is to clarify the prevalence of gallbladder benign neoplasms, their ultrasonographic appearance and their relationship with gallbladder lithiasis and cancer. METHODS: This study was carried out on 9000 consecutive patients having ultrasound of upper abdomen. Only adenomas and papillomas are considered as true benign neoplasms of the gallbladder. Adenomiomatosis and cholesterol polyps, often erroneously labelled as benign neoplasms, were excluded. Patients were followed-up by ultrasound every three months up to two years. RESULTS: The prevalence of benign neoplasms was 1.19%. Papillomas were found more frequently than adenomas both in males (68.51%) and in females (94.33%). Gallstones were not concomitant with benign neoplasms in any case. Neither stones nor growth of gallbladder benign neoplasms were recorded within the two-year follow-up period. CONCLUSIONS: Papillomas were more frequent than adenomas. No gallstone was concurrent with gallbladder benign neoplasms in our series. However, when gallstones are evidenced at ultrasound, further attention is recommended to discover probable concomitant neoplasms. Papillomas and adenomas more than 1 cm in diameter should be quarterly followed-up, while smaller masses could be six-monthly controlled. Surgery should be indicated for large-sized or rapidly growing masses because of the risk for cancer development.
Cytogenetic analyses of benign tumors in the past 2 decades have established consistent and recurrent abnormalities in a large proportion of these tumors. In particular, chromosomal analyses of meningiomas, adenomas of the salivary gland, uterine leiomyomas and lipomas have led to the identification of cytogenetic subtypes characterized by specific structural or numerical abnormalities. A comparison of these consistent changes shows a clustering of aberrations to specific regions on chromosomes 12, 1, 2, 6, 11, 13 and 22. The specificity of the involvement of these chromosome regions in the various benign neoplasms suggests that genes of importance in growth regulation rather than those related to malignant transformation are located at these sites.
Benign neoplasms of the maxillo-facial region require different treatment than do malignant lesions. Conservation of adjacent tissues is vital in order to optimize postoperative function and esthetics. The Le Fort I down-fracture technique is a historically proven approach for resecting benign neoplasms involving the nasal and para-nasal cavities. The Le Fort I, as practised today, is transoral and placed superior to the dento-alveolar processes of the maxilla. Surgical access, therefore, need not result in a skin scar nor loss of dental structures. Postoperative recovery to full function is rapid. The elegance and versatility of the Le Fort I down-fracture technique is discussed with reference to the report of a large, intra-antral benign odontogenic neoplasm.
To clarify the features of apocrine mixed tumors (AMT) of the skin among benign neoplasms with apocrine differentiation in their relationship to follicular stem cells, we investigated the immunohistochemical expression of CK15 (LHK15 and C8/144B), which is a relatively specific marker of hair follicle stem cells in the bulge, in 35 cases of eight different benign neoplasms with presumed apocrine differentiation. All eight cases of AMT of the skin showed CK15 immunostaining of the neoplastic cells, and all four cases of syringocystadenoma papilliferum, all five cases of spiradenoma, and both cases of cylindroma also showed a focally positive reaction to CK15. None of the other benign neoplasms with presumed apocrine differentiation showed CK15 expression. In AMT of the skin, the proportion of CK15-positive cells in the follicular or sebaceous differentiation group (78.8%, average of four cases) was significantly higher than the group without this differentiation (8.8%, average of four cases). AMT of the skin are unique among benign neoplasms with apocrine differentiation in their substantial and constant CK15 expression, suggesting that they derive from multipotent epithelial stem cells in the bulge. AMT of the skin with follicular or sebaceous differentiation are considered to show an immature stage of apocrine differentiation still rich in stem cells or to originate from stem cells with an incompletely established apocrine fate. The partially positive reaction for CK15 in syringocystadenomas papilliferum and spiradenoma/cylindroma may depend on the ability to express CK15 in stem cells with an apocrine fate or result from the follicular and apocrine nature of this neoplasm.
Giant esophageal polyp is a very rare neoplasm, usually benign but often demanding both for diagnostic and/or therapeutical procedures and for dramatic symptomatologic onset in the patient (regurgitation and asphyxia). The authors present a brief clinical report of a patient with a 20 cm.-long esophageal polyp removed by left lateral cervicotomy and esophagotomy for 8 cm. below the upper esophageal sphincter (histologic examinations showed a pedicled polypoid fibrolipoma with a remarkable presence of myxoid and vascular components). The authors also describe diagnostic and therapeutic methods to manage this rare disease and they gave a brief review of recent literature.
Benign neoplasms and cysts are the most common tumors of the mediastinum. However, they are still an infrequent clinical entity. Their diagnosis has been aided by the recent advances in CT scanning, MRI, ultrasonography, radionucleotide scanning, and fine-needle aspiration. The treatment of these benign tumors in children and the majority of adults is excision. In asymptomatic adults, observation or fine-needle aspiration and observation may be considered for pericardial cysts and selected neurogenic tumors and bronchogenic cysts.
This second part of our review about vascular proliferations summarizes the clinicopathologic features of the cutaneous vascular hyperplasias and benign neoplasms. Hyperplasias comprise a heterogeneous group of vascular proliferations that eventually show a tendency to regression. Angiolymphoid hyperplasia with eosinophilia is included within the group of hyperplasias because of its historical denomination and its reactive nature, probably as a consequence of an arteriovenous shunt, although usually the lesions do not regress. Pyogenic granuloma, bacillary angiomatosis, intravascular papillary endothelial hyperplasia, and pseudo-Kaposi's sarcoma qualify as vascular hyperplasias because they regress when the stimulus that initiated them is removed. Benign neoplasms form a large group of hemangiomas with distinctive clinicopathologic characteristics, although some of them are of recent description and may produce diagnostic difficulties. We classified cutaneous benign vascular neoplasms according to their cell lineage of differentiation, for example, endothelial, glomus cell, and pericytic differentiation. Subsequent categories are established according to the size of the involved vessels (capillaries, venules and arterioles, or veins and arteries) or the nature of the proliferating vessels (blood or lymphatic vessels). Capillary and cavernous hemangiomas have been the terms classically used to name the most common variants of benign vascular neoplasms (i.e., infantile hemangiomas), but they are not the most appropriate denominations for these lesions. First, these names are not contrasting terms. Furthermore, most of the socalled "cavernous" hemangiomas are not hemangiomas (neoplasms) at all, but venous malformations. The most important conceptual issue is that, at any point in time, a particular hemangioma has its own histopathologic pattern throughout the depth of the lesion. For these reasons, we classified hemangiomas into superficial and deep categories. Some of the lesions reviewed have been recently described in the literature, and they may histopathologically mimic lesions of Kaposi's sarcoma; these include targetoid hemosiderotic hemangioma, microvenular hemangioma, tufted hemangioma, glomeruloid hemangioma, kaposiform hemangioendothelioma, spindle-cell hemangioendothelioma, and benign lymphangioendothelioma. In each of these lesions, we update and emphasize those clinical and histopathologic features that are helpful for differential diagnosis with lesions of authentic Kaposi's sarcoma in any of its three stages of development (patch, plaque, or nodule).
Angiomyolipomas are benign tumors of the kidney derived from putative perivascular epithelioid cells, that may undergo differentiation into cells with features of melanocytes, smooth muscle, and fat. To gain further insight into angiomyolipomas, we have generated the first human angiomyolipoma cell line by sequential introduction of SV40 large T antigen and human telomerase into human angiomyolipoma cells. These cells show phenotypic characteristics of angiomyolipomas, namely differentiation markers of smooth muscle (smooth muscle actin), adipose tissue (peroxisome proliferator-activator receptor gamma, PPARgamma), and melanocytes (microophthalmia, MITF), thus demonstrating that a single cell type can exhibit all of these phenotypes. These cells should serve as a valuable tool to elucidate signal transduction pathways underlying renal angiomyolipomas.
Sebaceoma is a new term proposed to designate a distinctive benign neoplasm of adnexal epithelium with differentiation toward sebaceous cells. Clinicopathologic correlation of the condition is presented in a study of 14 cases. This neoplasm has many histologic features in common with trichoepithelioma and cylindroma, both of which are benign neoplasms of adnexal epithelium, one with follicle-like and the other with eccrine-like differentiation. Sebaceoma is a recently recognized entity, and the term is recommended to supersede, not merely to redefine, what has been previously called sebaceous epithelioma.
OBJECTIVE: An epidemiologic study of multiple primary malignant neoplasms i endometrial cancer patients is presented. DESIGN: From 1984-1996 121 endometrial carcinomas have been evaluated in the Department of Gynecology & Obstetrics in Hospital of Słupsk retrospectively. All double and triple neoplasms have been histologically recorded, doubtful cases have been excluded. MATERIAL & METHODS: Of 121 endometrial carcinomas 12 (9.9%) were multiple malignant neoplasms. Of these neoplasms 7 (5.8%) occur together with breast cancer and 5 (4.1%) with other primary malignomas, 11 are double, 1 is triple malignomas. During the 12-year period of study, 23 patients (19.0%) out of 121 patients diagnosed as having endometrial cancer had another primary benign neoplasms. Of these neoplasms 19 (15.7%) occur together with leiomyomas of uterus and 4 (3.3%) with ovarian cysts. The relation of endometriosis to endometrial carcinoma has been subject of only few studies. The investigations have resulted on the association between the conditions. Endomeriosis was found in association with endometrial carcinoma in 12 (9.9%) of 121 cases. In two cases of endometriosis carcinoma developed. CONCLUSION: There is not statistically significant difference in survival rate between group with synchronous primary malignant neoplasms and group with second primary benign neoplasms or group without multiple primary neoplasms (p = 0.07). Patients with endometrial cancer should be carefully and regularly followed up by monitoring at every anatomic site, especially the breast, stomach, and colon, in order that the development of a second primary carcinoma can be detected as early as possible, and not be overlooked in examinations.
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Benign urachal neoplasms have been rarely reported. We describe a case of a large benign mesenchymal neoplasm (21 x 19 x 14 cm) arising from the urachus, with imaging by computed tomography and ultrasound.