Epidermal inclusion cyst of the breast . A literature review

An epidermal inclusion cyst (EIC) of the breast is a rare, benign condition that may potentially be malignant. The present study conducted a systematic review of the literature in order to identify pathological hypotheses, clinical characteristics, and diagnostic and treatment options. A search for relevant studies was conducted through the Scopus, Embase and Medline databases during September 2014. The search term employed was ῾epidermal inclusion cyst breast᾽. Studies were selected if they contained adequate information regarding symptoms at presentation, diagnostic tools, pathology, characteristics, type of procedure performed and follow-up routines. A total of 35 papers describing 91 patients affected by EIC of the breast were identified. Following this, a total of 82 patients, including an additional case supplied from the present study, were selected for further analysis. EIC of the breast typically occurs during the fifth decade of life. A palpable mass of the breast was present in 65 (79%) patients. Ultrasonographic imaging was consistently utilized as a diagnostic tool in all the cases analyzed, whereas fine-needle aspiration cytology was used in 70% of the cases and mammography in 65%. No tumor recurrence was reported at a mean follow-up time of 53 months. The present study demonstrated that elliptical excision is the preferred treatment for EIC of the breast, with pathological analysis required to exclude malignancy

Peliosis hepatis. Personal experience and literature review

Peliosis hepatis (PH) is a disease characterized by multiple and small, blood-filled cysts within the parenchymatous organs. PH is a very rare disease, more common in adults, and when it affects the liver, it comes to the surgeon’s attention only in an extremely urgent situation after the lesion’s rupture with the resulting hemoperitoneum. This report describes the case of a 29-year-old woman affected by recurring abdominal pain. CT scans showed a hepatic lesion formed by multiple hypodense areas, which showed an early acquisition of the contrast during the arterial phase. Furthermore, it remained isodense with the remaining parenchyma during the late venous phase. We decided on performing a liver resection of segment Ⅶ while avoiding a biopsy for safety reasons. The histopathologic examination confirmed the diagnosis of focal PH. PH should always be considered in the differential diagnosis of hepatic lesions. Clinicians should discuss the possible causes and issues related to the differential diagnosis in addition to the appropriate therapeutic approach. The fortuitous finding of a lesion, potentially compatible with PH, requires elective surgery with diagnostic and therapeutic intents. The main aim is to prevent the risk of a sudden bleeding that, in absence of properly equipped structures, may have a fatal outcome

Duodenal gastrointestinal stromal tumors: Review on clinical and surgical aspects

Introduction: Gastrointestinal stromal tumors (GISTs) arising in the duodenum represent a rare entity and can be very demanding to manage. The diagnosis can be difficult (as these tumors can be misdiagnosed as pancreatic head tumors), and to treat owing to the complex anatomy of the duodenum and of the pancreatic head. Complete resection may require extensive procedures such as pancreaticodudodenectomy. Methods and results: An extensive literature review regarding clinico-pathological features, diagnostic investigations and surgical options in the management of duodenal GISTs has been performed. Conclusions: Duodenal GISTs have uncertain malignant potential, and they may be asymptomatic, or present with abdominal pain or bleeding. A preoperative diagnosis can be difficult to obtain. Endoscopic ultrasound and fine needle aspiration cytology (FNAC) may be helpful. Surgical R0 resection remains the only curative approach. However, owing to the complex anatomy of the duodenum, limited resection is not always feasible. In these cases, extensive procedures such as pancreaticoduodenectomy or pancreas-preserving duodenectomy are the treatment of choice. (C) 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved

Isolated Multifocal Sclerosing Thyroiditis: Case Report

Multifocal fibrosclerosis is a very rare fibroproliferative syndrome involving multiple organ systems. In our report we present the case of multifocal sclerosing thyroiditis characterized by multiple fibroelastic foci similar to breast “radial scar”, which can be misdiagnosed as multifocal papillary carcinoma. The diagnosis of multifocal sclerosing thyroiditis, in according with Armed Forces Institute of Pathology (AFIP) textbook on thyroid tumors, was made only after histological and himmunohistochemical examination. We consider the multifocal sclerosing thyroiditis as the first stage of multifocal IgG4-related sclerosing disease for the presence of IgG4 within fibrosclerotic tissue of thyroid. Total body CT-scan and plamatic IgG4 levels must be investigated in order to exclude or confirm the presence of systemic disease

A rare case of malignant epithelioid angiomyolipoma in multiple locations: Multifocal disease or metastases?

Background: Perivascular epithelioid cell tumors (PEComas), make up a family of extremely rare mesenchymal neoplasms, with characteristic morphological, immunohistochemical and molecular findings. Malignant PEComas and gastrointestinal epithelioid angiomyolipoma (E-AML) are especially rare. To the best of our knowledge E-AML have not been found in the breast. The difficulty in determining what constitutes optimal therapy for PEComas, owing to the sparse literature available, led us to report this rare case. Methods: We report a case of a 44-year-old woman, with a family history of multiple endocrine neoplasia syndrome (MEN) (gastrinoma, medullary thyroid cancer and parathyroid hyperplasia), affected by PEComa located in the kidney, stomach, ileum, liver and breast. Results: The renal, gastric, ileal and mammarian tumors were completely resected, with no evidence of local disease. Liver lesions were biopsied. The morphological and immunohistochemical findings confirm the diagnosis of PEComa. Conclusion: On this basis it is difficult to determine if some E-AML are multifocal tumors or metastatic disease

Surgical treatment of pheochromocytoma in MEN 2.

Multiple endocrine neoplasia type 2 (MEN 2) is a rare autosomal dominant cancer syndrome. Forty to fifty percent of patients with MEN 2A develops pheochromocytoma. Surgeons treating these patients with pheochromocytoma have always been faced with question of whether to perform mono-or bilateral adrenalectomy and the timing of surgical intervention. Over the past 20 years, thanks to the development of ever more sophisticated techniques of diagnostic imaging (TC, MRI, Scintigraphy, PET), which make it possible to identify small lesions, and to ever more rapid laboratory tests, there has been a change in the surgical management of this condition. Surgeons moved from bilateral open adrenalectomy (6- 9) to laparoscopic partial adrenalectomy and cortical sparing (10-13). After partial adrenalectomy one third of the patients require replacement therapy because the function of the residual parenchyma was compromised by excessive devascularization during surgery. In patients with bilateral pheochromocytoma it is advisable to perform only partial adrenalectomy of at least one gland, i.e. to completely remove the gland with the larger lesion and remove part of the gland with the smaller lesion to reduce the risk of recurrence. The authors report 4 cases of MEN 2, including 2 first-degree relatives, which illustrate the progress made in surgical treatment for pheochromocytoma

Retrocaval mass in patient with von Recklinghausen disease: case report.

Type I Neurofibromatosis (NF1) is an autosomal-dominant inheritable disorder, with an incidence of 1:3,000, and a prevalence of 1:4,000 to 5,000. Pathogenesis is based on mutations of the NF1 gene, a tumor suppressor gene encoding a cytoplasmic protein named neurofibromin that controls cellular proliferation. Patients affected by NF1 typically present with cutaneous neurofibromas, cafè au lait spots and eye involvement, but they can also be affected by various visceral tumors, such as neurofibromas (nodular or plexiform type), gastrointestinal stromal tumors or endocrine tumors, such as pheochromocytomas. Visceral neurofibromas are often asymptomatic but when growing in size they may present with pain, palpable abdominal mass, symptoms secondary to bowel obstruction or main vessels compression, and even gastrointestinal bleeding when mucosa or submucosa are involved. In these cases surgery becomes mandatory in order to remove all neoplastic tissue. The Authors describe a case of a young man affected by NF1 with associated retrocaval abdominal mass with compression and displacement of the inferior vena cava, thus requiring a complex surgical procedure

Vacuum-assisted closure treatment of leg skin necrosis after angiographic embolization of a giant plexiform neurofibroma.

Type 1 neurofibromatosis is a relatively common inherited disease of the nervous system, with a frequency of almost 1 in 3000. It is associated with neurofibromas of various sites. Our case report is about the surgical management of a giant neurofibroma of the right gluteal fold in a 46-year-old male with NF1. The patient presented with increasing edema and accelerated growth of the mass; he underwent percutaneous embolization of lesion vessels that induced necrosis of the neurofibroma. The patient was taken to the operating room, where surgical resection of the bulk of the lesion was undertaken. The postoperative course was complicated by delayed wound closure managed with antibiotics and vacuum-assisted wound closure. Giant neurofibromas similar to this tumor require complex preoperative, intraoperative and postoperative management strategies. Surgical debulk is best managed with preoperative percutaneous embolization that help to avoid surgical bleeding. Postoperative delayed wound closure was managed with the application of negative pressure in a closed environment that triggers granulation and tissue formation