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BACKGROUND: Benign subungual proliferation of the distal nail matrix and bed such as longitudinal keratosis, seborrhoeic keratosis or onychocytic matricoma should belong to a single spectrum of diseases. OBJECTIVE: This article intends to demonstrate clinically and histologically the different facets of Nail unit longitudinal acanthoma. METHODS: We report three new cases that present as a longitudinal melanonychia with thickening of the nail plate in two of them and as leukoxanthonychia in the third one. We compared them with the two original cases we described in 1999, reminiscent of seborrhoeic keratosis and all the new cases published since then. RESULTS: We therefore consider that all these tumours belong to a single spectrum of diseases, we have called 'nail unit longitudinal acanthoma' which describes a histopathological process. The distinct clinical features of these tumours, melanonychia or leukoxanthonychia may be linked to their variable anatomic locations in different zones of the nail unit.

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Clinical classification of onychomycosis is based on how the pathogenic agent penetrates the unguis. The disto-lateral sub-ungual variety is the most common. Dermatophytes (especially Trichophyton rubrum) and Scytalidium in tropical regions (Sc hyalium, Sc dimidiatum) are the most frequent toenail pathogens. Progression of a yellow friable sub-ungual hyperkeratosis associated with longitudinal striations and/or xanthonychial or leuconychial zones (sometimes pigmented with Trichophyton rubrum nigricans or Scopulariopsis brevicaulis) lead rapidly to onycholysis and later to total dystrophy of the ungual plate. Associated paronychia is more common in scytalidiasis. Candida species (tropicalis, parapsilosis) and fungi (Aspergillus sp. Fusarium sp, Acremonium sp, Penicillium sp, Scopulariopsis brevicaulis) generally colonize pre-existing onycholysis. For the fingernails, candidal colonizations secondary to pre-existing onycholysis is much more frequent than primary dermatophyte or scytalidium onychomycoses which are much less hyperkeratotic than on toenails. The one hand two feet tinea syndrome caused by Trichophyton rubrum is a particular entity. Proximal sub-ungual onychomycoses without fingernail or toenail paronychia is generally caused by Trichophyton rubrum in immunodepressed subjects (AIDS). Initial proximal leuconychia progresses to the distal part of the nail. Proximal lesions associating proximal leuconychia and paronychia result from fungi, Fusarium being the most commonly identified agent. Onyxis complicating chronic paronychia, generally related to Candida colonization, occurs in subjects with particular conditions (immunodepression, distal vascular disorders). Superficial onychomycosis, e.g. superficial toenail leuconychia, is mainly due to Trichophyton interdigitale, more exceptionally to Trichophyton rubrum (children, immunodepressed), and rarely Candida (children). Endonyx onychomycosis occurs when the pathogen invades the entire thickness of the nail (milky leuconychia without sub-ungual hyperkeratosis). Trichopnyton violaceum or soudanense is the most common pathogen. Even though the clinical presentation of onychomycosis is highly suggestive of the pathogenic agent, the lack of specific criteria implies a mycological sample to confirm the diagnosis and identify the causal agent before initiating treatment.

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BACKGROUND: Distal digital keratoacanthoma (DKA) is an uncommon tumor difficult to diagnose clinically, and even histologically, with certainty. OBJECTIVE: Our purpose is to report on two new cases and to discuss the clinical, histologic, and differential diagnosis. METHODS: We have reviewed all well documented cases published in the literature. RESULTS: No single diagnostic criterion is sufficiently sensitive and specific to be pathognomonic. CONCLUSION: The diagnosis of DKA should be based on the correlation of clinical, radiological and pathologic findings, but the tumor is frequently diagnosed histologically as "squamous cell carcinoma, keratoacanthoma type" or as keratocarcinoma.

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OBJECTIVE: Very little has been published on longitudinal melanonychia in children. Our objective was to determine the nature of melanocytic lesions in pediatric patients with longitudinal or total melanonychia and to look for correlations between clinical and histologic features. METHODS: All patients younger than 16 years of age with longitudinal or total melanonychia who were evaluated at our nail disorder outpatient clinic between September 1993 and September 1996 were included. The clinical and histologic features of the nail condition were determined in each case. RESULTS: Forty patients were included. The final diagnosis was nevus in 19 cases (junctional in 17 cases and compound in 2), lentigo in 12 cases, and functional longitudinal melanonychia in 9. The latter corresponded to a hyperpigmentation caused by melanocytic activation with no increase in the number of melanocytes. None of the patients had melanoma. Appearance within the first year of life, periungual pigmentation, and total melanonychia were consistent features in patients with melanocytic hyperplasia (lentigo or nevus). Early onset of a dark broad lesion in a white patient was typical of melanocytic hyperplasia, although none of these features were pathognomonic. CONCLUSION: Benign melanocytic hyperplasia (lentigo or nevus) was the cause of 77.5% of cases of longitudinal melanonychia in our overall pediatric population and of 85% of cases in the subset of white patients. All the remaining cases of longitudinal melanonychia were the result of melanocytic activation.

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BACKGROUND: Forty-nine cases of circumferential pharyngolaryngectomy with total esophagectomy (PLTE) done between 1982 and 1996 were studied retrospectively. These procedures were performed for advanced squamous cell tumors of the superior esophageal sphincter (n = 23), for hypopharyngeal tumors with synchronous esophageal carcinoma (n = 15), and for hypopharyngeal tumors extensively invading the cervical esophagus (n = 11). METHODS: Ninety-six percent of the patients had T3-4 lesions, and it was impossible to use a free jejunal graft reconstruction. Patients underwent primary surgery in 70% of the cases, and salvage surgery (after failure of chemoradiotherapy) in 30%. In most patients, esophagectomy was performed without thoracotomy (n = 45). Resection was curative (R0) in 70% of the cases, in spite of lymph node invasion in 94%. Reconstruction of the digestive tract was achieved with the stomach in 33 patients (67%) or with the colon in 16 patients (33%). RESULTS: Before 1989, postoperative mortality was high, was correlated with the high frequency of palliative surgery, and resulted in unsatisfactory survival results (overall 5-year survival rate of 7%). After 1989, as a result of better selection of patients and appropriate training of our team, postoperative mortality decreased from 33% to 10%, R1-2 resections decreased from 39% to 26%, and a 3-year overall survival rate of 28% was obtained for the last 25 patients, all of whom were able to eat without difficulty. These results are superior to the survival rates and functional results obtained with radiochemotherapy alone for such advanced tumors, even though the voice is preserved with radiochemotherapy alone. CONCLUSIONS: PLTE for advanced pharyngeal or cervical esophageal tumors is the best treatment currently available, but it is indicated only in very selected cases: when it is technically impossible to perform reconstruction with a free jejunal graft after circumferential pharyngolaryngectomy; as primary surgery, rather than as salvage surgery following chemoradiotherapy; after careful preoperative morphologic and endoscopic assessment of the extent of the tumor; and in patients able to tolerate a thoracotomy for an esophagectomy with lymphadenectomy. Selection according to these guidelines should improve results.

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BACKGROUND/AIMS: Liver tumors may be unresectable for volumetric reasons; the post-hepatectomy future remaining liver (FRL) will be too small to ensure survival. In some cases, preoperative selective portal vein embolization (PSPVE) of the tumorous part of the liver can permit the induction of hypertrophy of the FRL and convert patients from an unresectable to a resectable status. METHODOLOGY: Analysis of the efficiency of PSPVE in changing the volume of the FRL and in permitting curative hepatectomy was performed in a retrospective study of 28 initially unresectable (for volumetric reasons), consecutive cases treated from September 1987 to September 1995. Fifty percent of the cases had damaged liver parenchyma. PSPVE was performed in various locations, according to the site of the tumor and impairment of the liver parenchyma. RESULTS: Twenty-five PSPVE (89%) successfully induced sufficient hypertrophy of the FRL. Explanations could be found retrospectively for the 3 failures. For the 28 cases, the mean increase in the FRL was 70%, and the mean ratio between the FRL and the whole functional liver changed from 21.5% before PSPVE, to 33.9% after PSPVE. Twenty-three patients could be hepatectomized (82%). CONCLUSIONS: With this technique, liver tumors considered to be unresectable, due to life-threatening volumetric insufficiency, may be considered resectable lesions, and there is an increase in the safety of some extended hepatectomies. These good results were mainly due to application of the distal and proximal free flow embolization technique, with non-absorbable material, and perhaps to the long interval of one month between PSPVE and hepatectomy. Indications in normal liver parenchyma are for patients with a very small left lobe or those requiring a right hepatectomy with wedge resections of the left liver. Indications for damaged liver parenchyma also include some cases requiring left trisegmentectomy or central hepatectomy.

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The prognosis of peritoneal carcinomatosis (PC), classically treated with intravenous chemotherapy, is very poor (mean survival of 6 months). The aim of this study is to report the results of a phase II study in which PC was treated with complete cytoreductive surgery, with the residual microscopic disease treated by immediate intraperitoneal post-operative chemotherapy (IIPC) for 5 days (mitomycin with fluorouracil or Adriamycin with Platinol. Fifty-four patients with PC from miscellaneous origins were treated between January 1993 and April 1996. The PC was important (clinically evident) but with no extraperitoneal localization in 29 cases. The PC was moderate or minor in 25 cases having been fortuitously discovered during a laparotomy for extraperitoneal cancer localization. Operating time was 7:21 h, associated frequently with extensive peritonectomies, and with resection of invaded organs (four organs per patient). IIPC was complete (5 days) in 91 per cent of patients. Three post-operative deaths (5.5%) occurred. Morbidity was present in 61 per cent of patients, and was related to surgical extension (P < 0.001). A 2-year survival of 50% was mainly correlated with the importance of the PC (P < 0.01), and was the same for both groups of patients (isolated major PC vs moderate (or minor) PC associated with extraperitoneal localization). PC recurrence rates were 30 per cent at 2 years. Complete cytoreductive surgery associated with IIPC is a logical and promising treatment of moderate and minor PC. However, it appears that it is a heavy treatment for patients (and physicians), and its efficacy will be proved only after a randomized study for survival, quality of life, and cost (currently ongoing).

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OBJECTIVES: The short term prognosis of peritoneal carcinomatosis whose classical treatment is intravenous chemotherapy, is poor (mean survival of 6 months). The aim of this study was to report the results of a phase II prospective study in which peritoneal carcinomatosis was managed with complete reductive surgery associated with treatment of the residual microscopic disease with immediate intraperitoneal postoperative chemotherapy. PATIENTS AND METHODS: Fifty-four patients with peritoneal carcinomatosis from miscellaneous origins, were treated between January 1993 and April 1996. Major peritoneal carcinomatosis was important, clinically evident, but without extraperitoneal localization in 29 cases. It was moderate, fortuitously discovered during a laparotomy for an extraperitoneal recurrence in 25 cases. Immediate intraperitoneal postoperative chemotherapy was carried out continuously during 5 days, with 900 ml/m2 of ringer lactate, with either mitomycine C and 5-fluorouracil, or doxorubicin and platinum, according to histology. The treatment was complete in 91% of cases. RESULTS: Three patients died during the hospitalization (5.5%), and a high morbidity (61%) was observed, with 35% intra-abdominal complications necessitating surgery in 13% of the patients. The postoperative complications were correlated with the extension of the cytoreductive surgery (P < 0.001). After a mean follow-up of 12.3 months, 13 patients died. The 2-year survival rate was 50%. Survival was related to the importance of the peritoneal carcinomatosis (P < 0.01) and was identical for patients with isolated peritoneal carcinomatosis and for patients with moderate peritoneal carcinomatosis associated with resected extra-peritoneal disease. The incidence of recurrence of peritoneal carcinomatosis was 30% at 2 years, showing the efficiency of this new procedure to treat peritoneal carcinomatosis. CONCLUSIONS: Complete cytoreductive surgery with immediate intraperitoneal postoperative chemotherapy is a promising treatment of peritoneal carcinomatosis. However it appears that: a) it is a difficult treatment for patients and for physicians, b) its efficiency will be asserted only with a randomized study (currently ongoing), that only allows to suppress selection bias, c) it is able to cure some groups of peritoneal carcinomatosis (probably 20%), that will be difficult to identify, and d) improvement of immediate intraperitoneal postoperative chemotherapy is possible (mainly with hyperthermia). The main advantage of immediate intraperitoneal postoperative chemotherapy is that, after proving its efficiency, easy widespread use will be assured.

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Thirty-two selected patients underwent laparotomy in an attempt to resect one or more isolated liver metastases (LM) from breast cancer. Only 21 of them had hepatectomy and systematic lymph node picking of the hepatic pedicle. In six patients (19%), the discovery of diffuse metastatic disease contraindicated hepatectomy and in five patients (16%), the diagnosis of LM was erroneous, for lesions proved to be benign liver tumours. Nineteen of the resected cases received preoperative chemotherapy, 12 received post-operative chemotherapy and two had repeated hepatectomy. Eight patients (38%) had more than one LM and (24%) had positive hepatic lymph nodes. No post-operative mortality occurred. After the beginning of this combined treatment, median survival was 38.2 months and 2- and 5-year survival rates were, respectively, 78% and 24%. After the hepatectomy, median survival was 26 months and 2- and 5-year survival rates were, respectively, 50% and 9%. When a recurrence did occur (mean time to recurrence after hepatectomy was 14.8 months) the liver was involved in 75% of the cases and was the first place of recurrence in 56% of the patients. In this limited series, the number of LM, the number of positive pedicular lymph nodes, and a response to preoperative chemotherapy were not significant prognostic factors. However, patients with negative nodes tended to have a better prognosis, as did those with the first and only site of relapse being the liver. These selected patients, treated with hepatectomy, had a median survival at least three-fold that of patients treated with standard, non-surgical treatment. However, hepatectomy appeared to be mainly a cytoreductive procedure, and the efficiency of this combined treatment was mainly hampered owing to the inefficacy of current chemotherapy programmes. Only a prospective randomized study, in well-defined patients with isolated LM from breast cancer, comparing conventional treatment with or without hepatectomy, will demonstrate whether hepatectomy does indeed increase survival rates.

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