Primary umbilical endometriosis: Surgical case report.

Introduction: Endometriosis is a benign nevertheless a chronic condition which impacts greatly the quality of life through cyclic discomfort. We aim to report the case of umbilical endometriosis and a literature review of the different treatment modalities. Case Report: This was a case of a 43-year-old woman, with no history, who presented with a painful hemorrhagic umbilical swelling during the menstrual period associated with dysmenorrhea. Abdominal ultrasound revealed a subcutaneous umbilical mass of non-vascularized tissue nature confirmed on Doppler. Pelvic MRI which confirms the diagnosis of primary umbilical endometriosis. The patient underwent wide local excision of the endometriotic nodule with umbilical reconstruction. Histology confirmed the diagnosis of umbilical endometriosis. Resection margins were clear. Discussion: Extra-pelvic endometriosis sites are not common, especially the umbilicus. It usually occurs secondary to surgical scars, specifically after laparoscopy or open abdominal surgery. Surgical management is currently described as gold standard. Laparoscopic approach is recommended as it allows better visual inspection for secondary localization of endometriosis. Medical management corresponds to combined oral contraceptives (COCs) or progestins for management of endometriotic implants decreasing inflammatory effects, or Gonadotropin-releasing hormone for long-course treatment. Malignant transformation of the umbilical nodule has been described in literature with a reported risk of malignant transformation to be 3%. Conclusion: Current management of extragenital endometriosis suggest radical surgery with wide local excision. Due to the rarity, there is a paucity of data on umbilical endometriosis and mostly reported from case reports.

Systemic paraneoplastic vasculitis secondary to papillary carcinoma of the thyroid.

Systemic vasculitis secondary to thyroid carcinomas is exceptional. We report the case of a 55-year-old woman who presented with systemic vasculitis involving leucocytoclastic cutaneous vasculitis and renal disorders secondary to papillary thyroid carcinoma (PTC). Her symptoms resolved completely after total thyroidectomy. Other causes of vasculitis were excluded. To our knowledge, this is only the second case reported of systemic vasculitis associated with PTC in a paraneoplastic manner.

[Tumoral calcinosis]. / Calcinose tumorale d'Inclan.

BACKGROUND: Tumoral calcinosis is a rare disease seen in adolescents and young adults and consisting of calcifications in periarticular soft tissue. Mutations in fibroblast growth factor 23 and GalNAc transferase 3 have been identified in the familiar forms of tumoral calcinosis. PATIENT: A 10 year-old boy of light phototype presented multiple calcified periarticular masses since the age of seven years as well as sciatica secondary to nerve compression. Laboratory examinations revealed hyperphosphoraemia. Serum calcium levels, kidney tests, parathormone assay and muscle enzymes were all normal. Histology of a cutaneous biopsy sample revealed dermal calcifications. Inclan's tumoral calcinosis was diagnosed. Surgical resection was performed and drug treatment comprising phosphate chelators (aluminium hydroxide) was administered. At five months, other sites were apparent. DISCUSSION: This case of tumoral calcinosis is notable in terms of its sporadic onset in a child of light phototype and by the presence of compression of the external sciatic nerve. The diagnosis was made after ruling out other causes of soft tissue calcification.

[Skin cancer and psoralen plus UVA: a retrospective study of 106 patients exposed to a great number of PUVA treatments]. / PUVAthérapie et cancers cutanés: etude rétrospective chez 106 malades ayant reçu des doses élevées de PUVA.

INTRODUCTION: The benefits of PUVAtherapy in many dermatological affections are well known. Its carcinogenic role in the long term has been assessed varyingly in American and European series. OBJECTIVE: The aim of this study was to assess the role of PUVA in the onset of cancers of the skin. METHODS: Retrospective study of patients presenting with psoriasis and followed-up in the phototherapy unit of the Michallon Hospital in Grenoble since 1976 and having received more than 150 sessions of PUVA. The parameters studied were: age, gender, phototype, age at the time of the first irradiation, type of phototherapy administered, total number of sessions, concomitant treatments, administration of retinoids and the appearance of skin cancers with the interval before their onset after the first session, their localization and their histological type. RESULTS: One hundred six patients were retained among the 152 who replied to the inclusion criteria. Having died or been lost to follow-up, forty-six patients were excluded. Fourteen patients had presented at least one cutaneous tumor with a total number of 35. Excluding the keratoacanthomas, 13 patients had a non-melanic cutaneous cancer with a total number of 32 tumors. Ten out of the 14 were phototype III, 3 were phototype II and one was phototype IV. Nine out of 14 had received PUVAtherapy alone and 5 PUVAtherapy and broad spectrum UVB. The number of sessions of PUVA received in all the cases was more than 200 (220 to 780), corresponding to a total dose of UVA comprised between 1460 and 3882 Joules. The delay before onset of the tumors varied from 6 to 27 years after the first PUVAtherapy. The mean age at the time of the first irradiation was of 50.2 years (14-75 years). The mean duration of phototherapy was of 10 years (2.23 years).

[Heller's myotomy without fundoplication: a series of 123 patients]. / L'opération de Heller sans système anti-reflux. A propos de 123 cas.

INTRODUCTION: Surgical treatment of achalasia of lower oesophageal sphincter is Heller's myotomy, usually associated with a fundoplication due to an high risk of postoperative gastro-oesophageal reflux. The value of this fundoplication is discussed. The aim of this study was to evaluate retrospectively the results of Heller's myotomy without fundoplication but performed according to a precise technique preventing postoperative reflux. PATIENTS AND METHODS: Between 1975 and 1999, 123 patients underwent Heller's myotomy without systematic fundoplication. Diagnosis of achalasia was performed clinically and confirmed by investigations: baryum meal, fibroscopy and manometry. Myotomy was performed through an abdominal approach in 117 (95%) patients. Dissection preserved fixity of abdominal oesophagus in all cases, particularly its posterior meso. Myotomy was performed on abdominal oesophagus but not below the cardia. Posterior fundoplication was associated in 2 patients. RESULTS: One patient (0,8%) died from massive aspiration. Morbidity (1,6%) consisted in one peritonitis and one postoperative occlusion. At follow-up (mean = 5 years; range: 1-20), functional results were satisfying (excellent and good) in 112 (92%) patients. Seven patients (6%) developed postoperative reflux, including one who need surgical treatment. Dysphagia persisted in 3 patients (2%) who had to be reoperated. CONCLUSION: Results of this series show that systematic fundoplication is not necessary in Heller's myotomy for achalasia of lower oesophageal sphincter.

[Leiomyoma of the esophagus]. / Léiomyome de l'oesophage.

BACKGROUND: Leiomyoma is a benign tumor rarely occurring in the esophagus. Only 1% of esophageal tumors are leiomyomas. CASE REPORT: A 70-year-old man underwent surgery for an asymptomatic tumor of the thoracic esophagus. The tumor was enucleated via right thoracotomy and histology confirmed the diagnosis of leiomyoma. Outcome was favorable. DISCUSSION: Leiomyomas of the esophagus generally develop from the muscularis and exceptionally from the muscularis mucosae, producing a parietal tumor or a pediculated endoluminal tumor respectively. Surgery is indicated for all cases, with the possible exception of asymptomatic leiomyoma. Encleation can be achieved by videothoracoscopy. Resection of the esophagus can be discussed if enucleation is impossible for giant tumors, or ring tumors at the esogastric junction, or if the mucosae wound cannot be repaired. Endoscopic resection is indicated for pediculated leiomyoma. Malignant transformation is exceptional but an association between esophageal myomatosis and carcinoma of the esophagus is possible. Certain authors thus advocate resection for all cases, even small asymptomatic leiomyomas.

[Adenocarcinoma of the anal glands]. / Adénocarcinome des glandes anales.

The study aim was to report an adenocarcinoma of the anal glands revealed by an anal abscess in a 64-year-old man. Malignant transformation of an anal fistula is discussed in the genesis of this disease. Clinical symptoms are not specific. Abdomino-perineal resection of the rectum is the usual surgical treatment and adjuvant radiation therapy didn't prove its efficiency.

[Inflammatory pseudotumor of the spleen]. / Pseudotumeur inflammatoire de la rate.

Inflammatory pseudotumor of the spleen is a very rare benign lesion of unknown etiology. Splenectomy is recommended to obtain histological diagnosis and to eliminate a malignant tumor.