Subsegmental approaches to S7: anatomic laparoscopic transdiaphragmatic and nonanatomic robotic transthoracic.

BACKGROUND: Minimally invasive liver surgery of postero-superior segments (S4a, S7, S8) remains a challenge. The caudal view, an increased distance between trocars and the operative field, and the liver fulcrum limiting the view, contribute to the difficulty [1, 2]. We and other groups have previously reported the use of intercostal trocars to access subdiaphragmatic tumors (transdiaphragmatic approach) [3-5], only few reports on a laparoscopic total transthoracic approach, none (to our knowledge) dynamic manuscripts of a total transthoracic robotic approach, and none (to our knowledge) that use preoperative port site and anatomic modelling exist. Further, we developed a total transthoracic (thoracoscopic) approach to avoid a hostile abdomen, while bringing viewing axis and instruments close to the target [6-10]. In this context, this report details the advantages of a laparoscopic vs. robotic transthoracic approach. According to institutional protocol, reports of individual cases in print or video format do not require institutional review board approval. PATIENT: A 68-year-old male on peritoneal dialysis with left colon adenocarcinoma and a single synchronous liver metastasis in S6-7 close to the root of the right hepatic vein underwent a laparoscopic transdiaphragmatic metastasectomy. Two years later, the patient developed a recurrent 1.5 cm liver metastasis in S7, which lend itself to a robotic transthoracic approach. TECHNIQUE: Following 3-D modelling and virtual port placement planning, the first metastasectomy was performed laparoscopically using a transdiaphragmatic approach. The recurrence was managed transthoracically due to more apical, subdiaphragmatic location. For this operation, a robotic approach was optimal as robotic wrist articulation facilitates manipulation via the limited intercostal space. This was particularly helpful during the diaphragmatic reconstruction. CONCLUSIONS: Total transthoracic liver surgery is certainly an advanced procedure requiring superior MIS liver skills. Recommendations for starting with a total transthoracic approach are not unlike from starting a standard, none-transthoracic liver surgery. Early on in the experience we recommend advanced liver MIS skills, and single, small, subdiaphragmatic tumors away from major vessels. Nonetheless, when these recommendations are followed a total transthoracic approach may be safer and result in less access trauma, than traversing a hostile abdomen to reach the posterior-superior liver. Both laparoscopic and robotic transthoracic approaches can facilitate the resection of subdiaphragmatic tumors, especially in patients with hostile abdomens. While the laparoscopic approach has advantages due to a broader spectrum of available surgical tools (flexible tip camera, parenchymal dissection, and energy devices), the robotic wrist articulation facilitates manipulation via the restricted intercostal space.

Small Intestinal Adenocarcinoma in a Patient With Celiac Disease.

Celiac disease (CD) is a systemic immune-mediated disorder against gluten, leading to an autoantibody response causing damage to the small intestinal mucosa. CD has been associated with gastrointestinal malignancies, most commonly gastrointestinal lymphoma. Rare malignancies have also been reported, such as small intestinal adenocarcinoma. In this report, we present a case of a 91-year-old male with a history of CD, noncompliant with a gluten-free diet, who presented with weight loss, abdominal pain, and gastrointestinal bleeding secondary to a newly discovered adenocarcinoma of the jejunum.

Metastatic Syringocystadenocarcinoma Papilliferum: A Case Report, Tumor Genomic Profiling, and Literature Review.

Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare cutaneous neoplasm of the apocrine or eccrine sweat glands. Solid and cystic glandular structures with cribriform and tubular architecture along with CK5/6, pankeratin and p63 immuno-profile set apart SCACP from other cutaneous malignancies. Wide local excision (WLE) has been the mainstay treatment for localized SCACP; however, no standard treatment has yet been established for unresectable or metastatic disease. Herein, we report a 74-year-old male with SCACP, who initially presented with a painful nodule on the upper back and later developed metastatic disease. He was treated with carboplatin and paclitaxel with concurrent intensity-modulated radiation therapy (IMRT), which resulted in disease stabilization for 12 months. Next generation sequencing (NGS) revealed a total of 18 genomic alterations associated with potential benefit from targeted therapeutics. PD-L1 expression was identified in 70% of tumor cells. These findings suggest that the opportunity of targeted therapeutics and immunotherapy exist as for metastatic SCACP. Reporting molecular profile of the rare tumors with no established standard treatment options should be encouraged.

ROS1-rearranged lung adenocarcinoma with peritoneal carcinomatosis on initial presentation.

Peritoneal carcinomatosis (PC) is progression of the primary cancer to the peritoneum that is seen in only 1.2% of patients with lung cancer. It is associated with poor prognosis especially if present at the time of initial cancer diagnosis. The predisposing factors for peritoneal spread are not yet well understood. It has been suggested that the oncogene status of the tumour can influence the patterns of metastatic spread. There is not enough data about the role of c-ROS oncogene 1 (ROS1) mutation in the development of PC in non-small cell lung cancer. Here, we describe a case of a 56-year-old man who presented with new-onset ascites and was found to have PC. He was diagnosed with ROS1-rearranged lung adenocarcinoma. No obvious primary tumour was identified. Patient responded well to targeted therapy with crizotinib and remained 6 months free of disease progression.

Regional metastatic paraganglioma of the bladder: a rare cause of myocardial infarction, reversible cardiomyopathy and an intracardiac thrombus.

Sympathetic paragangliomas are rare neuroendocrine tumours that arise from chromaffin cells and secrete catecholamines. On rare occasions, patients with sympathetic paragangliomas can present with symptoms of congestive heart failure. The optimal treatment is surgical to remove all disease and thereby improve survival as well as restore cardiac function. We report a case of a patient with a regional metastatic bladder paraganglioma and a succinate dehydrogenase complex subunit B gene mutation presenting with cardiomyopathy who had significant improvement in his cardiac function with surgical resection despite further progression of metastatic disease. During his 4-year follow-up period, the patient remains free from heart-failure signs and symptoms.

Rituximab for retroperitoneal fibrosis due to IgG4-related disease: A case report and literature review.

Retroperitoneal fibrosis (RPF) is a progressive fibroinflammatory disease that can be complicated by urinary obstruction. RPF can be the only manifestation of IgG4-related disease (IgG4-RD). Treatment of IgG4-related RPF is challenging and mostly consists of long-term glucocorticoids leading to significant side effects and treatment intolerance. Recent exploration of the role of rituximab as a B-cell depleting therapy in the treatment of IgG4-RD provides therapeutic potential as a well-tolerated alternative to glucocorticoids. We present a case of IgG4-related RPF for which rituximab was instituted as a steroid-sparing treatment strategy. Following 4 doses, kidney function partially recovered, and the disease went into remission. We discuss the potential merit of rituximab for the treatment of patients with IgG4-related RPF.

An unusual presentation of xanthogranulomatous pyelonephritis: psoas abscess with reno-colic fistula.

Xanthogranulomatous pyelonephritis (XGP) is a rare histological subset of pyelonephritis characterized by being a chronic destructive granulomatous inflammation of the renal parenchyma. XGP is classified according to the extent of disease into two entities: within the renal cortex (focal or segmental XGP) or diffuse spread with pelvic communication (diffuse XGP). Although rare, XGP can have fatal complications including perinephric, psoas abscess, nephro-cutaneous fistula and reno-colic fistula. Only few studies have reported XGP complicated with psaos abcess and reno-colic fistula. Our aim is to add to the literature and share our experience with a case of extensive XGP eroding into the psoas muscle and ascending colon leading to severe sepsis that was successfully managed. We report a 56-year-old woman who was found to have XGP complicated by psoas abscess and reno-colic fistula managed by antibiotics, nephrostomy, and subsequent nephrectomy and partial colectomy.

Therapeutic non-toxic doses of TNF induce significant regression in TNFR2-p75 knockdown Lewis lung carcinoma tumor implants.

Tumor necrosis factor-alpha (TNF) binds to two receptors: TNFR1/p55-cytotoxic and TNFR2/p75-pro-survival. We have shown that tumor growth in p75 knockout (KO) mice was decreased more than 2-fold in Lewis lung carcinoma (LLCs). We hypothesized that selective blocking of TNFR2/p75 LLCs may sensitize them to TNF-induced apoptosis and affect the tumor growth. We implanted intact and p75 knockdown (KD)-LLCs (>90%, using shRNA) into wild type (WT) mice flanks. On day 8 post-inoculation, recombinant murine (rm) TNF-α (12.5 ng/gr of body weight) or saline was injected twice daily for 6 days. Tumor volumes (tV) were measured daily and tumor weights (tW) on day 15, when study was terminated due to large tumors in LLC+TNF group. Tubular bones, spleens and peripheral blood (PB) were examined to determine possible TNF toxicity. There was no significant difference in tV or tW between LLC minus (-) TNF and p75KD/LLC-TNF tumors. Compared to 3 control groups, p75KD/LLC+TNF showed >2-5-fold decreases in tV (p<0.001) and tW (p<0.0001). There was no difference in tV or tW end of study vs. before injections in p75KD/LLC+TNF group. In 3 other groups tV and tW were increased 2.7-4.5-fold (p<0.01, p<0.0002 and p<0.0001). Pathological examination revealed that 1/3 of p75KD/LLC+rmTNF tumors were 100% necrotic, the remaining revealed 40-60% necrosis. No toxicity was detected in bone marrow, spleen and peripheral blood. We concluded that blocking TNFR2/p75 in LLCs combined with intra-tumoral rmTNF injections inhibit LLC tumor growth. This could represent a novel and effective therapy against lung neoplasms and a new paradigm in cancer therapeutics.

Chronic low dose-rate radiation down-regulates transcription related to mitosis and chromosomal movement similar to acute high dose in prostate cells.

PURPOSE: Despite concerns over risks from exposure to low-dose ionizing radiations encountered in the environment and workplace, the molecular consequences of these exposures, particularly at representative doses and dose-rates, remains poorly understood. MATERIALS AND METHODS: Using a novel flood source construct, we performed a direct comparison of genome-wide gene expression regulations resulting from exposure of primary human prostate fibroblast cultures to acute (10 cGy and 200 cGy) and longer-term chronic (1.0-2.45 cGy cumulative over 24 h) exposures. RESULTS: Expression profiling showed significant differential regulation of 396 genes with no measureable changes in the acute 10 cGy dose. However, there were 106 genes in common between samples given an acute 200 cGy dose compared to those given chronic doses, most of which were decreased and related to cell cycle or chromosomal movement in M-phase. Biological pathway analysis showed decreases in cell cycle, chromosomal movement, cell survival and DNA replication, recombination and repair as well as a predicted activation of transcriptional regulators TP53, RB1 and CDKN2A. In agreement with these results, prostate epithelial cells given 200 cGy or chronic doses displayed functional decreases in proliferation and mitotic cells. CONCLUSIONS: In summary, we showed a contrast to the common observation of constant or reduced effect per unit dose as the dose (acute) was diminished, that even very low total doses delivered chronically could rival the perturbing effect of acute doses 100 times as intense. Underscored is the importance of the means of dose delivery, shown to be as important as dose size when considering biologic effect.

Divergent modification of low-dose 56Fe-particle and proton radiation on skeletal muscle.

It is unknown whether loss of skeletal muscle mass and function experienced by astronauts during space flight could be augmented by ionizing radiation (IR), such as low-dose high-charge and energy (HZE) particles or low-dose high-energy proton radiation. In the current study adult mice were irradiated whole-body with either a single dose of 15 cGy of 1 GeV/n 56Fe-particle or with a 90 cGy proton of 1 GeV/n proton particles. Both ionizing radiation types caused alterations in the skeletal muscle cytoplasmic Ca²âº ([Ca²âº]i) homeostasis. 56Fe-particle irradiation also caused a reduction of depolarization-evoked Ca²âº release from the sarcoplasmic reticulum (SR). The increase in the [Ca²âº]i was detected as early as 24 h after 56Fe-particle irradiation, while effects of proton irradiation were only evident at 72 h. In both instances [Ca²âº]i returned to baseline at day 7 after irradiation. All 56Fe-particle irradiated samples revealed a significant number of centrally localized nuclei, a histologic manifestation of regenerating muscle, 7 days after irradiation. Neither unirradiated control or proton-irradiated samples exhibited such a phenotype. Protein analysis revealed significant increase in the phosphorylation of Akt, Erk1/2 and rpS6k on day 7 in 56Fe-particle irradiated skeletal muscle, but not proton or unirradiated skeletal muscle, suggesting activation of pro-survival signaling. Our findings suggest that a single low-dose 56Fe-particle or proton exposure is sufficient to affect Ca²âº homeostasis in skeletal muscle. However, only 56Fe-particle irradiation led to the appearance of central nuclei and activation of pro-survival pathways, suggesting an ongoing muscle damage/recovery process.

Exostoses of the paranasal sinuses.

While bony exostoses of the external auditory canal have long been recognized as a complication of cold-water swimming, bony exostoses of the paranasal sinuses have not been previously reported. We present an unusual case of multiple exostoses of the paranasal sinuses, which began coincident with nasal irrigation with cold gentamicin solution. The patient had had prior endoscopic sinus surgery. Topical antibiotic gentamicin irrigation lessened recurrence of bacterial sinus episodes. He admitted to using the irrigation directly from the refrigerator daily for two years. A biopsy was consistent with exostosis. Patients should be counseled to avoid irrigating their paranasal sinuses with cold irrigants. Laryngoscope, 2010.

Diagnostic utility of CD10, CD3 and electron microscopy of renal cortical neoplasms with oncocytic features.

No currently defined imaging techniques can reliably distinguish between oncocytoma and epithelial malignant lesions with oncocytic features in the kidney; therefore, patients must undergo resection or, in certain circumstances, biopsy to definitively establish diagnosis. Immunohistochemical staining for CD10 and CD3, evaluation of the staining pattern and intensity, and relevant morphologic appearance are helpful diagnostic tools in discriminating between renal cell carcinoma with oncocytic features and renal oncocytoma. Electron microscopy confirms different ultrastructural components of each neoplastic cell type and correlates with immunohistochemical findings. Accurate determination of the tumor origin would allow for the use of limited nephron sparing and laparoscopic surgical approaches to treat appropriately.

Micropapillary urothelial carcinoma: clinico-pathologic review.

Micropapillary carcinoma is a rare distinct variant of high grade urothelial carcinoma, which has specific morphological characteristics and is almost always associated with muscularis propria and vascular invasion. No currently defined imaging techniques can reliably diagnose some types of deeply invasive urothelial carcinoma of urinary bladder, in particular its micropapillary variant. Therefore, the pathological findings are crucial in making the diagnosis. Micropapillary carcinoma (MPC) is a tumor with an aggressive clinical course, an advanced stage of disease at the time of presentation, and usually a poor outcome. Metastatic micropapillary carcinoma to bladder should always be included in the differential diagnosis. Correct histological diagnosis of this aggressive neoplasm would allow timely, albeit intense, radical treatment of the disease. The current most generally favored treatment option for all patients who present with MPC is immediate radical cystectomy.

Synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney: a unique case report and review of the literature.

BACKGROUND: Malignant transformation of mature cystic teratoma is a rare complication. While any of the constituent tissues of a teratoma has the potential to undergo malignant transformation, squamous cell carcinoma is the most commonly associated malignancy. Renal carcinoid tumors are rare and frequently associated with horseshoe kidney and renal teratoma. Renal teratoma rarely presents together with carcinoid tumor or adenocarcinoma. To the best of our knowledge, there has never been a report of renal teratoma coexisting with both carcinoid tumor and adenocarcinoma. METHODS: Here, we present a unique and first case of synchronous primary carcinoid tumor and moderately differentiated adenocarcinoma arising within mature cystic teratoma of horseshoe kidney in a 50-year-old female. Lumbar spine X-ray, done for her complaint of progressive chronic low back pain, accidentally found a large calcification overlying the lower pole of the right kidney. Further radiologic studies revealed horseshoe kidney and a large multi-septated cystic lesion immediately anterior to the right renal pelvis with central calcification and peripheral enhancement. She underwent right partial nephrectomy. RESULTS: Macroscopically, the encapsulated complex solid and multiloculated cystic tumor with large calcification, focal thickened walls and filled with yellow-tan gelatinous material. Microscopically, the tumor showed coexistent mature cystic teratoma, moderately differentiated adenocarcinoma and carcinoid tumor. Immunohistochemically, alpha-methylacyl-coenzyme A-racemase, calretinin, CD10 and thyroid transcription factor-1 were negative in all the three components of the tumor. The teratomatous cysts lined by ciliated epithelium showed strong staining for cytokeratin 7 and pancytokeratin, and those lined by colonic-like epithelium showed strong staining for CDX2, cytokeratin 20 and pancytokeratin, but both were negative for calretinin. Additionally, the teratomatous cyst wall showed strong staining for smooth muscle actin, and weak staining for carbonic anhydrase IX, CD99, chromogranin and synaptophysin. The adenocarcinoma component was strongly positive for cytokeratin 7 and pancytokeratin, weakly positive for synaptophysin and CD56, and negative for carbonic anhydrase IX, CD99, CDX2, chromogranin, cytokeratin 20 and smooth muscle actin. The carcinoid tumor component was strongly positive for CD56, chromogranin and synaptophysin, weakly positive for pancytokeratin, and negative for carbonic anhydrase IX, CD99, CDX2, cytokeratin 7, cytokeratin 20 and smooth muscle actin. She received no adjuvant therapy and is alive without evidence of disease six months after diagnosis and surgery. CONCLUSION: This unique and first case herein presented with synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney emphasizes the need for thorough sectioning and entire submission for histologic evaluation of mature cystic teratomas, in order to avoid missing multiple additional histogenetically distinct neoplasms.

Case of a concurrent renal mass and extragonadal retroperitoneal teratoma.

The presentation of a synchronous renal cell carcinoma (RCC) and germ cell tumor (GCT) is rare. We report the case of a 57 year-old male who presented with a right renal mass and retroperitoneal lymphadenopathy. He underwent a successful right partial nephrectomy and retroperitoneal lymph node dissection, and the subsequent pathology revealed a stage I clear cell RCC and a retroperitoneal teratoma with a component of benign prostatic tissue. We briefly discuss the rarity of this occurrence, the pathological features that helped support this diagnosis, and the likely etiologies of these synchronous lesions.