Primary cutaneous anaplastic large cell lymphoma with angioinvasive features and cytotoxic phenotype: a rare lymphoma variant within the spectrum of CD30+ lymphoproliferative disorders.

BACKGROUND: Primary cutaneous anaplastic large cell lymphoma (PCALCL) presents with solitary or grouped exophytic tumors and cohesive infiltrates of large CD30+ T cells. OBJECTIVE: To report an angioinvasive variant of PCALCL. METHODS: Retrospective analysis of clinicopathological features of this variant. RESULTS: The group consisted of six patients (median age 46 years) with a solitary flat necrotic lesion preferentially located on the upper extremity. Histologically, there were angiocentric and angiodestructive infiltrates of medium-sized to large pleomorphic and anaplastic cells co-expressing CD30 and CD8. Five patients were treated with surgical excision and one patient with radiotherapy. A relapse was observed in one patient with spontaneous regression of the lesions suggesting a link to the recently described angioinvasive lymphomatoid papulosis (type E). All patients were alive without evidence of disease after a median follow-up of 31 months (range 15-96), indicating an excellent prognosis. CONCLUSIONS: The angioinvasive variant of PCALCL is rare but distinctive and prone to misinterpretation as aggressive lymphoma due to its histological features.

Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC).

BACKGROUND: Granulomatous cutaneous T-cell lymphomas (CTCLs) are rare and represent a diagnostic challenge. Only limited data on the clinicopathological and prognostic features of granulomatous CTCLs are available. We studied 19 patients with granulomatous CTCLs to further characterize the clinicopathological, therapeutic, and prognostic features. OBSERVATIONS: The group included 15 patients with granulomatous mycosis fungoides (GMF) and 4 with granulomatous slack skin (GSS) defined according to the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. Patients with GMF and GSS displayed overlapping histologic features and differed only clinically by the development of bulky skin folds in GSS. Histologically, epidermotropism of lymphocytes was not a prominent feature and was absent in 9 of 19 cases (47%). Stable or progressive disease was observed in most patients despite various treatment modalities. Extracutaneous spread occurred in 5 of 19 patients (26%), second lymphoid neoplasms developed in 4 of 19 patients (21%), and 6 of 19 patients (32%) died of their disease. Disease-specific 5-year survival rate in GMF was 66%. CONCLUSIONS: There are clinical differences between GMF and GSS, but they show overlapping histologic findings and therefore cannot be discriminated by histologic examination alone. Development of hanging skin folds is restricted to the intertriginous body regions. Granulomatous CTCLs show a therapy-resistant, slowly progressive course. The prognosis of GMF appears worse than that of classic nongranulomatous mycosis fungoides.

Comparison of ultrasound-guided core-needle biopsy and fine-needle aspiration in the assessment of head and neck lesions.

BACKGROUND: Core-needle biopsy (CNB) has been successfully applied in other medical specialties, but its value is undetermined in otolaryngology. METHODS: This prospective study includes 75 patients, who were seen at our institution with a cervical mass. The results of CNB were compared with those of fine-needle aspiration (FNA) in the 68 histologically verified cases. RESULTS: CNB was superior to FNA providing a specific diagnosis (90% vs 66%) and achieved a higher accuracy in identifying true neoplasms (100% vs 93%) and detecting malignancy (99% vs 90%). However, the sensitivity and specificity did not differ significantly between both methods. CONCLUSIONS: Sonography and if necessary FNA should continue to be the investigation method of first choice for head and neck lesions. The main indication for CNB is after repeated failures of FNA to provide a diagnosis. It can also be performed in patients who are not surgical candidates or in those who refuse surgery.

Primary extramedullary plasmacytoma: similarities with and differences from multiple myeloma revealed by interphase cytogenetics.

Primary extramedullary plasmacytoma is an indolent neoplasm that infrequently converts to multiple myeloma. Since cytogenetic data on extramedullary plasmacytoma are lacking, we studied 38 cases of this type of neoplasm by fluorescence in situ hybridization. Fourteen cases (37%) contained IGH breaks, including six with a t(4;14) translocation. No translocations t(11;14), t(14;16), t(8;14), nor breaks involving MALT1, BCL6 or FOXP1 were found. Loss of 13q (40%), as well as chromosomal gains (82%) were common. There was no correlation between chromosomal alterations and clinical features or local relapse. Cytogenetically, extramedullary plasmacytoma and multiple myeloma are closely related. However, the distribution of IGH translocation partners, with the notable absence of t(11;14), is different. Key words: extramedullary plasmacytoma, multiple myeloma, cytogenetics, IGH translocation, fluorescence in situ hybridization.

Is silicone oil optic neuropathy caused by high intraocular pressure alone? A semi-biological model.

BACKGROUND: Silicone oil endotamponade is used for the repair of complicated retinal detachments. Cataract, glaucoma and corneal endothelial dysfunction are the most frequent complications of silicone oil tamponade. Clinical and histopathological studies have revealed that silicone oil can penetrate into the optic nerve and into the brain. The mechanism by which silicone oil moves from intraocular into the optic nerve is still under debate. To investigate the effect of intraocular pressure only, a post-mortem experimental histological study was performed to determine whether silicone oil penetration from the globe into the optic nerve after vitrectomy and silicone oil instillation is a purely pressure-related phenomenon. Although a post-mortem study excludes physiological processes, it serves as a model for the study of pure physical forces onto biological structures. METHODS: The study was carried out on 20 human eyes with their optic nerves attached. All specimens had been harvested from patients without known eye disease. The vitreous body was removed with a syringe and the globe was filled with silicone oil. A lipophil fluorescence marker (Bodipy) was added in 8 eyes. The mean intraocular pressure after silicone oil filling measured 40 mm Hg and the globes stayed under pressure for up to 16 weeks. The eyes and optic nerves were stained with H&E and examined with light, phase-contrast and fluorescence microscopy. RESULTS: None of the 20 specimens examined showed silicone oil in the retrolaminar portion of the optic nerve. CONCLUSIONS: Migration of silicone oil into the optic nerve was not demonstrated in this human post-mortem study. Therefore other factors, such as pre-existing glaucomatous damage to the disc region and/or active transport mechanisms must be involved in the development of silicone oil-associated optic neuropathy.

The anatomy of the muscle insertion (scleromuscular junction) of the lateral and medial rectus muscle in humans.

PURPOSE: To analyze the histologic features of the insertion of the medial and the lateral rectus muscles in humans. METHODS: Postmortem study performed on 49 extraocular muscles from 21 subjects without known ocular disease. All muscles were obtained no longer than 8 hours after death, after consent for autopsy. Thirty-seven lateral recti muscles and 12 medial recti muscles were studied with light microscopy (hematoxylin-eosin and Goldner stains) as well as with enzyme histochemistry and immunohistochemistry, with monoclonal-human tenascin C antibody. RESULTS: Light microscopic studies of muscle insertions of the lateral and the medial rectus muscle demonstrated muscle tissue connecting directly to the sclera without a tendon. These findings were confirmed immunohistochemically with tenascin C-antibody staining. CONCLUSIONS: Based on the results of this postmortem study in humans the term "muscle tendon" should be used with caution for the insertional area (scleromuscular junction) of the lateral and medial extraocular muscles. Light microscopy, histochemistry, and immunohistochemistry demonstrate that the tissue at the scleromuscular junction contains striated muscle with minimal connective (tendinous) tissue connecting to the sclera. To the best of the authors' knowledge, this is the first study in which enzyme histochemistry and immunohistochemistry have been used to investigate the anatomy of the insertional area (muscle-tendon-sclera junction) of the extraocular muscles in humans.