1/682. Pulmonary lymphangiomyomatosis: three new cases studied with electron microscopy. Three cases of pulmonary lymphangiomyomatosis are described, with emphasis on the ultrastructural changes. The clinicopathologic features corresponded to those previously described. Each patient was a female in the reproductive years; breathlessness and recurrent pneumothoraces were the predominant clinical features. Histologically, the lungs showed a focal interstitial infiltrate of short, spindle-shaped mononuclear cells compatible with primitive smooth muscle, which was associated with irregular emphysema and hemosiderosis. Electron microscopy confirmed the smooth muscle nature of the pulmonary infiltrate and showed the presence of cells intermediate between smooth muscle and fibroblasts. Abnormalities were also noted in the pulmonary connective tissue that are possibly related to the fragility of the lung in this condition. ( info) |
| A case is reported of severe transient macroglossia after biopsy from the tongue in a 13-year-old boy who has had intermittent macroglossia since the age of 1 year as a result of extensive lymphangioma circumscriptum of the tongue. The acute lesions appear to result from haemorrhage into the lymphatic spaces following rupture of blood vessels in connective tissue septa and possibly secondary infection. ( info) |
3/682. Acquired vulvar lymphangioma mimicking genital warts. A case report and review of the literature. A 44-year-old female developed confluent, dusky red, pruritic labial papules clinically suspected to be genital warts. She had a long-standing history of Crohn's disease with vulvar fistulae. The papular eruption developed after several bouts of cellulitis in a region of vulvar lymphedema. Shave biopsy of a papule exhibited papillated epidermal hyperplasia overlying a dermis with a 'Swiss-cheese' appearance secondary to lymphedema and superficial ectatic thin-walled vascular spaces characteristic of lymphangiectasias. review of published cases reveals that acquired lymphangiomas often affect the vulva compared to other cutaneous sites and can be associated with surgery, radiation therapy, infection (e.g., erysipelas, tuberculosis), Crohn's disease, congenital dysplastic angiopathy and congenital lymphedema. Rather than translucent vesicles ('frog spawn') typical of extragenital cutaneous lymphangiomas, vulvar lymphangiomas often present as verrucous papules that can be mistaken for genital warts. In this case, we believe that the combination of vulvar Crohn's disease and recurrent cellulitis resulted in local lymphatic destruction, lymphedema and ultimately symptomatic lymphangiectasias that mimicked genital warts. ( info) |
4/682. A case of intra-abdominal multiple lymphangiomas in an adult in whom the immunological evaluation supported the diagnosis. A 60-year-old patient with intra-abdominal lymphangiomatosis is described. He presented with anaemia due to enteric haemorrhage, hypoproteinaemia with heavy hypogammaglobulinaemia and T-cell lymphopenia. Duodenal biopsy showed lymphangiectasia while a small bowel study revealed several filling defects in the terminal ileum. On exploratory laparotomy, numerous inoperable lymphangio-haemangiomata were found, involving the small and large intestine, appendix, mesenterium, gallbladder and main biliary tract. The importance of T-cell lymphopenia and hypogammaglobulinaemia in the diagnosis of intra-abdominal lymphangiomatosis with lymphangiectasia is stressed. ( info) |
5/682. Fine needle aspiration cytology of a cervical lymph node lymphangioma in an adult. A case report. BACKGROUND: Although the cytologic features of cervical cystic lesions are well established, no cytology reports on lymphangioma in adults have been published. CASE: A 60-year-old male presented with a slowly growing, upper laterocervical, painless enlargement. Fine needle aspiration (FNA) obtained 15 mL of yellowish fluid, consisting predominantly of a uniform population of small and round lymphocytes without mitosis or atypia and with some histiocytes intermingled with them. Some centrocytes and occasionally centroblasts and plasma cells could also be observed. immunohistochemistry performed on cell block sections displayed polyclonal B lymphocytes mixed with T cells. The specimen showed a clearly circumscribed, 50-mm, cystic lesion with a multilocular appearance and abundant, yellowish liquid. Microscopic examination demonstrated cystic lymphangioma arising from the medullary portion of a lymph node. CONCLUSION: FNA cytology permits a suggested diagnosis of lymphangioma. This is one of the few reports of FNA cytology of lymphangioma and, to the best of our knowledge, this entity has not previously been found as a neck mass in an adult. ( info) |
| Spontaneous chylothorax associated with primary lymphedema is an exceedingly rare clinical entity. We report a case of chylothorax in a 38-year-old woman with a history of primary lymphedema of the right leg and an inguinal lymphangioma. Pathophysiology, diagnosis, treatment, as well as literature review are presented. ( info) |
| lymphangioma is an extremely rare cause of swelling of the scrotum. In this case report we present the finding of such a tumor in a 20-year-old man. The clinical, ultrasonic, and pathological findings are described and the differential diagnoses of acute and chronic swelling of the scrotum are discussed. ( info) |
| Eleven cases of colonic lymphangioma, including 3 that have not been reported previously, are reviewed. Radiologic analysis reveals that 9 presented as polypoidal mucosal lesions and only 2 had characteristic features at a submucosal lesion. A predominant occurence in females (70%) and during the sixth and seventh decades are noted. ( info) |
9/682. Lymphangiomatous macroglossia. Lymphangiomatous macroglossia, or giant tongue, usually presents within the first two years of life. The tongue enlarges to the point of protrusion from the mouth with resultant ulceration and frank necrosis of the tip. There may be associated malocclusion and prognathia produced by the enlargement of the tongue. The pathology and clinical manifestations of lymphangioma of the tongue are discussed in this paper, and a case report is presented. ( info) |
10/682. Benign lymphangiomatous papules of the skin following radiotherapy: a report of five new cases and review of the literature. AIMS: The aim of this report is to summarize the clinicopathological findings of five cases of a pseudosarcomatous vascular proliferation of the skin at the site of radiotherapy following surgery for carcinoma of the breast and carcinoma of the endometrium. To our knowledge, only five cases of this presumably rare pseudomalignant proliferation have been reported previously. methods AND RESULTS: All patients were females ranging in age from 44 to 70 years. The lesions appeared as solitary or multiple papules or vesicles localized to the field of radiation. Microscopically, they were composed of vascular spaces that exhibited atypical features without qualifying for a diagnosis of angiosarcoma. CONCLUSIONS: Our cases provide additional evidence supporting the benign nature of this atypical vascular proliferation, not recurring, never developing metastases and being cured readily by local excision. Clinical, histopathological and ultrastructural findings suggest a lymphatic origin. Whether these lesions represent a neoplastic or a reactive condition secondary to radiotherapy is unclear. The name 'benign lymphangiomatous papules of the skin following radiotherapy' is proposed. ( info) |