Trousseau's Syndrome in Cholangiocarcinoma: The Risk of Making the Diagnosis.

We report a case of Trousseau's syndrome with cholangiocarcinoma complicated by a fatal pulmonary embolism after liver biopsy. A 69-year-old man who presented with right upper quadrant pain was found to have portal vein thrombosis and nonspecific liver hypodensities after imaging by computerized tomography. Following four days of anticoagulation, heparin was held for percutaneous liver biopsy. After the biopsy, he developed acute hepatic failure, acute kidney injury, lactic acidemia, and expired. Autopsy revealed intrahepatic cholangiocarcinoma and a pulmonary embolism. Trousseau's syndrome with cholangiocarcinoma is rarely reported and has a poor prognosis. This case highlights a fundamental challenge in the diagnosis and early management of intrahepatic cholangiocarcinoma with hypercoagulability. Diagnostic biopsy creates an imperative to reduce post-operative bleeding risk, but this conflicts with the need to reduce thrombotic risk in a hypercoagulable state. Considering the risk of withholding anticoagulation in patients with proven or suspected cholangiocarcinoma complicated by portal vein thrombosis, physicians should consider biopsy procedures with lesser bleeding risks, such as transjugular liver biopsy or plugged percutaneous liver biopsy, to minimize interruption of anticoagulation.

CT-guided percutaneous administration of Spot sterile carbon stain to a single F-18 FDG positive mesenteric lymph node to allow identification during subsequent laparoscopic resection.

PURPOSE: To evaluate the feasibility and efficacy of CT-guided percutaneous administration and off-label use of Spot sterile carbon stain (GI Supply, Camp Hill, PA) to a single PET-positive mesenteric lymph node to allow identification during subsequent laparoscopic resection. METHODS: An asymptomatic 61-year-old male veteran with past medical history only for benign prostatic hyperplasia presented with a mildly elevated PSA. A screening CT demonstrated a 2.3 × 0.8 cm mesenteric mass and findings resembling mesenteric panniculitis; however, following PET revealed F-18 FDG avidity, and a neoplastic process could not be entirely excluded. An initial attempt at percutaneous biopsy was non-diagnostic. Therefore, a second percutaneous intervention with localization and off-label administration of Spot dye was performed to aid in subsequent laparoscopic resection. RESULTS: The Spot dye was identified during laparoscopy and the PET-positive mesenteric mass was successfully resected. Histology diagnosed the non-malignant etiology of organizing hematoma. A follow-up PET/CT confirmed interval resection of the previous PET-avid mass. CONCLUSIONS: This case report is the first to describe the off-label administration and use of Spot dye by a CT-guided percutaneous technique, allowing improved visualization during subsequent laparoscopic resection. Spot is composed of sterile, carbon-based particles which leave a permanent tattoo and is FDA-approved for the inking of the Gastrointestinal tract. Interventional radiologists should consider the off-label use of Spot dye in marking regions of interest as it safely provides a permanent tattoo which may aid in future identification.

Marjolin's ulcer of the leg secondary to nonhealing chronic venous stasis ulcer.

We report on a 79-year-old man with a chronic venous stasis ulceration of >20 years' duration in the left medial leg presenting to our clinic after admission for bleeding and suspected infection of his ulceration. This lesion had been biopsied in 1999 and was found to be a benign, chronic venous insufficiency ulceration. Plain film radiographs as well as a bone scan did not identify osteomyelitis. Because of the hypertrophic, nodular appearance of this ulceration, it was the clinicians' discretion to perform a repeat biopsy of the lesion. Biopsy revealed invasive, well-differentiated squamous cell carcinoma. A computed tomography scan subsequently identified a suspicious inguinal lymph node, and an ultrasound-guided needle aspiration revealed metastatic squamous cell carcinoma of the biopsied lymph node. Above-knee amputation was indicated at this time and was performed in conjunction with local superficial inguinal lymph node resection, after which the patient was discharged from our service.

A 49-Year-Old Woman Presenting With Cavitary Lung Lesions: A Case Report.

Granulomatosis with polyangiitis (GPA) can be a challenging diagnosis to confirm due to significant overlap with other small-vessel vasculitis syndromes and similar presentations to non-vasculitic processes such as infection or malignancy. We report a case of a 49-year-old woman presenting with several months of cough, chest wall pain, and weight loss, who was found to have several cavitary lung lesions on imaging, no renal involvement, and unusual anti-neutrophil cytoplasmic antibody (ANCA) serologies. After tissue biopsy, the patient was diagnosed with GPA and treated with steroids and rituximab with clinical improvement at follow-up. Due to a complex clinical course and non-classic symptomatology, her diagnosis of GPA was not discovered until several months after symptom onset. Clinicians should consider GPA in the case of progressive cavitary lung lesions even in the absence of renal involvement or positive ANCA serologies, as prompt tissue diagnosis is crucial to allow for early initiation of treatment.

Percutaneous endopyeloplasty: a novel technique.

BACKGROUND AND PURPOSE: Despite a 10% to 15% failure rate, endopyelotomy remains the treatment of choice for most patients with ureteropelvic junction (UPJ) obstruction. We present a novel technique of percutaneous endopyeloplasty, wherein a precise, full-thickness approximation of a standard longitudinal endopyelotomy incision is performed in a horizontal Heineke-Mikulicz fashion through the conventional solitary percutaneous tract via a nephroscope. We assess the feasibility and efficacy of percutaneous endopyeloplasty in a chronic porcine bilateral UPJ obstruction model and compare outcome data with those#10; of conventional endopyelotomy and laparoscopic pyeloplasty. MATERIALS AND METHODS: Partial UPJ obstruction was created in 20 kidneys (11 pigs) by laparoscopic ligation of the upper ureter over a 5F ureteral catheter. After development of hydronephrosis over a period of 4 to 6 weeks, percutaneous endopyeloplasty (N = 10), conventional percutaneous endopyelotomy (N = 5), or laparoscopic pyeloplasty (N = 5) was performed. The essential steps of percutaneous endopyeloplasty include retrograde ureteral catheterization, standard percutaneous endopyelotomy incision, mobilization of the distal ureteral lip, horizontal suturing of the endopyelotomy incision through the nephroscope, and nephrostomy drainage and ureteral stenting. Suturing was performed using a modified 5-mm laparoscopic device (Sew Right 5 SR; LSI Solutions, Rochester, NY), which was passed through the nephroscope. RESULTS: Percutaneous endopyeloplasty was technically successful in all 10 kidneys with a mean total operative time of 81.4 minutes (range 51-117 minutes). The mean endopyeloplasty suturing time was 29.4 minutes (range 20-64 minutes). Three kidneys required two sutures, while seven kidneys required three sutures to complete the endopyeloplasty. The solitary complication was a lower-pole infundibular stenosis. Over a mean follow-up of 7.7 weeks, all renal units showed relief of obstruction, as evidenced by regression of hydronephrosis,#10; improvement in T(1/2) and glomerular filtration rate on renogram, and a low intrapelvic pressure on Whitaker test. At autopsy, the endopyeloplasty site showed a fine, well-healed transverse scar with no evidence of residual suture on the mucosal surface. The mean caliber of the UPJ following endopyeloplasty (13.8F +/- 2.2F) was significantly greater (P = 0.01) than that following endopyelotomy (7.5F +/- 1.9F). Intraoperative extravasation on completion of endopyeloplasty was absent (N = 6) or mild (N = 4) compared with that seen in all five kidneys following endopyelotomy. CONCLUSION: Percutaneous endopyeloplasty is feasible, simple, reproducible, and effective. Its advantages over conventional endopyelotomy include transrenal performance of a Fenger-plasty, wider caliber of the UPJ, absence of extravasation, and shorter duration of ureteral stenting.

Pagetoid squamous cell carcinoma in situ (pagetoid Bowen's disease) of the external genitalia.

Approximately 5% of cutaneous squamous cell carcinomas in situ (SCCIS) have a nested pattern, referred to as pagetoid SCCIS, or pagetoid Bowen's disease. This growth pattern may simulate extramammary Paget's disease (EPD) when the external genitalia are involved. We report two cases of genital pagetoid SCCIS, including the first example affecting the vulva. Using several known cases each of classic (bowenoid) SCCIS of the vulva, primary cutaneous vulvar EPD, and cutaneous melanoma in situ as controls, we performed a battery of immunohistochemical and mucin stains to study the phenotype of pagetoid SCCIS. Both cases of pagetoid SCCIS were strongly positive for cytokeratin (CK) 7, a highly sensitive and popular marker for EPD. Stains with the high molecular weight cytokeratin marker Keratin-903 (34betaE12) showed 4+ immunoreactivity, although not with the intensity seen in the juxtaposed normal keratinocytes or in the cells of classic SCCIS. Immunoreactivity scores were 2+ for CK5/6, 2+ for CK19, and 1+ to 3+ for CK 13. Unlike the Paget's cells of EPD, the nested cells of pagetoid SCCIS were devoid of mucin and were nonimmunoreactive with GCDFP-15, CEA, CAM5.2, and c-erbB2. Stains for CK20, S-100, and Melan A also were negative. Although CK7 is a sensitive marker for the Paget's cells of EPD, this study corroborates the unexpected CK7 positivity in pagetoid SCCIS, precluding its usefulness in distinguishing these two diseases. Combined with mucin stains, a limited immunohistochemical panel may be of diagnostic value in particularly difficult cases.

Genital tract tumors in Proteus syndrome: report of a case of bilateral paraovarian endometrioid cystic tumors of borderline malignancy and review of the literature.

Proteus syndrome is a rare, sporadic disorder that causes postnatal overgrowth of multiple tissues in a mosaic pattern. Characteristic manifestations include: overgrowth and hypertrophy of limbs and digits, connective tissue nevus, epidermal nevus and hyperostoses. Various benign and malignant tumors and hamartomas may complicate the clinical course of patients with the syndrome. Commonly encountered tumors include hemangiomas, lymphangiomas and lipomas. Tumors of the genital tract occur less often. Bilateral ovarian cystadenomas are regarded as having diagnostic value in Proteus syndrome when occurring within the first two decades of life. We describe a 3-year-old girl with Proteus syndrome who developed bilateral paraovarian villoglandular endometrioid cystadenomatous tumors of borderline malignancy (low malignant potential) of the broad ligament. Desmoplastic tumor implants, presumably noninvasive, were present in biopsies from the pelvic floor, cul-de-sac and omentum. This is the first recognized example of a cystic borderline epithelial tumor of the female genital tract and the first paraovarian tumor reported in a patient with Proteus syndrome. Previously reported tumors and cystic lesions involving the female genital tract and the male genital tract in patients with Proteus syndrome are reviewed. We suspect that specific testicular and paratesticular tumors may prove to have the same diagnostic value in Proteus syndrome as do bilateral cystic ovarian and paraovarian tumors.