Whipple disease.

Whipple disease (WD) is a rare disease caused by Tropheryma whipplei. The classic profile of the patient is that of a middle-aged man presenting with fever, chronic diarrhea, and arthralgias. Extragastrointestinal manifestations are not rare. A high degree of clinical suspicion for the disease is needed in atypical cases. Trimethoprim-sulfamethoxazole is the treatment of choice. We present two patients with WD. The first presented with melena and generalized hyperpigmentation. The second had depression for two years before the typical symptoms. Both hyperpigmentation and long-lasting depression without the typical manifestations of the disease are rare. Histologic examination of tissue biopsies was diagnostic for WD. Both patients were treated successfully with trimethoprim-sulfamethoxazole.

Generalized purpuric lichen nitidus. Report of a case and review of the literature.

Generalized purpuric lichen nitidus is an extremely rare form of lichen nitidus. We present the third documented case of this uncommon dermatosis, which was treated successfully with orally administered cyclosporine. During a 12-month follow-up after the cessation of the drug, no recurrence was noticed.

Tubular adenoma of the breast: a rare presentation and review of the literature.

UNLABELLED: Tubular adenomas, also known as pure adenomas, are rare epithelial tumors of the breast. Only a few cases have been reported in the literature, especially in young women of reproductive age. Postmenopausal women are very rarely affected. We describe here a very rare case of tubular breast adenoma in a postmenopausal woman who presented with a gradually enlarging breast lump. Clinical examination and imaging studies revealed a non-tender well circumscribed left breast tumor suggestive of a fibroadenoma. Due to the history of progressive enlargement of the breast lump, a surgical excision was performed. Histological findings were suggestive of a tubular breast adenoma. We conclude that although tubular breast adenoma is rare, it should always be considered in the differential diagnosis in postmenopausal patients presenting with a gradually enlarging breast mass. Preoperative diagnosis is difficult because tubular adenoma is indistinguishable from a fibroadenoma on physical examination and breast imaging. Surgical excision is necessary to establish a definitive diagnosis. Clinical presentation and management of our patient are discussed along with a review of the literature. KEYWORDS: Tubular adenoma; Breast; Breast mass.

Synchronous occurrence of neuroendocrine colon carcinoma and hairy cell leukemia.

UNLABELLED: BACKGROUND-PURPOSE: The risk of secondary malignancy development in patients with hairy cell leukemia has been evaluated in several studies with varying results. The aim of this study is to describe a case of synchronous occurrence of neuroendocrine colon carcinoma and hairy cell leukemia. METHODS: A 69-year-old man presented with rectal bleeding. Colonoscopy revealed a rectal tumor, whereas biopsy specimens revealed a poorly differentiated carcinoma. During the preoperative evaluation, pancytopenia was detected. RESULTS: At laparotomy, a mass was detected 16 cm from the anal verge and an anterior resection of the rectum was performed. Detailed histological and immunohistochemical analyses revealed a poorly differentiated neuroendocrine carcinoma of the rectum. Postoperative evaluation of pancytopenia revealed hairy cell leukemia. The patient was initially treated with chemotherapy for hairy cell leukemia followed by chemotherapy for neuroendocrine colon carcinoma. Survival was 44 months. CONCLUSIONS: To our knowledge, synchronous occurrence of neuroendocrine colon carcinoma and hairy cell leukemia has not been previously reported in the literature. Given the rare incidence of both entities in the general population, it is highly unlikely that they occurred together by chance. Further research is needed to determine what would be the optimal management options of patients with simultaneous hairy cell leukemia and a neuroendocrine colon cancer.

Breast cancer associated with type 1 neurofibromatosis.

The association between breast cancer and type 1 neurofibromatosis (NF1) is a rare clinical entity. We herein present the case of a 59-year-old woman, with typical clinical manifestations of NF1, who presented with a painless lump in her right breast, which she had first noticed 8 months earlier. Clinical examination and diagnostic workup were suggestive of a breast carcinoma, and a modified radical mastectomy was performed. Histopathological examination revealed a poorly differentiated invasive ductal breast carcinoma and multiple neurofibromas. The pathological staging was pT2N1a according to TNM/UICC. Delayed presentation of the patient was the result of her mistakenly identifying the breast tumor as a manifestation of NF1 neurofibromatosis.

Oncocytoma of the lacrimal gland diagnosed initially by fine-needle aspiration cytology.

Oncocytomas are benign tumors that infrequently involve ocular adnexa. We describe a case of oncocytoma of the lacrimal gland which was initially diagnosed by fine-needle aspiration cytology. An 83-year-old patient presented to the cytology laboratory with a peripunctal mass in the right eye. The tumor was aspirated. The cytoplasm of the tumor cells was rich in eosinophilic granules. The initial cytological diagnosis was oncocytoma. The tumor was then completely excised and the histological diagnosis confirmed the initial cytological one. Oncocytoma is a rare entity which must be considered in differential diagnosis of a peripunctal mass in elderly patients. The literature was reviewed confirming the rarity of such a presentation and the novelty of this case. Cytological criteria of malignancy are not yet established.

Survivin expression as a strong indicator of recurrence in urothelial bladder cancer. Predictive value of nuclear versus cytoplasmic staining.

BACKGROUND: Since its discovery in 1997, survivin has garnered significant interest due to its putative role as an inhibitor of apoptosis. Few studies have investigated the immunohistochemical status of survivin in urothelial bladder carcinoma. The subcellular localization of survivin (nuclear, cytoplasmic) and its differential predictive value is a parameter that previous studies have almost ignored. The aim of this study was to investigate the expression pattern of survivin in order to determine its potential prognostic significance. MATERIALS AND METHODS: Archival tumor tissue from 80 patients with urothelial carcinoma were analysed by immunohistochemistry. RESULTS: Nuclear and cytoplasmic positive scores of 61.25% (49/80) and 22.5% (18/80), respectively were found. Nuclear positive staining correlated strongly with increased grade (p=0.001), stage (p=0.039) and the probability of tumor recurrence (p=0.029). No relationship was found between the cytoplasmic survivin level and the clinicopathological parameters. Nuclear expression was identified as a significant independent predictor of relapse-free survival (p=0.016). CONCLUSION: Nuclear expression of survivin reflects an adverse disease outcome.