Outcomes of a Phase II Study of Intraperitoneal Paclitaxel plus Systemic Capecitabine and Oxaliplatin (XELOX) for Gastric Cancer with Peritoneal Metastases.

BACKGROUND: Adding intraperitoneal paclitaxel (IP-PTX) to paclitaxel/5-fluoropyrimidine has shown promising results in patients with gastric cancer peritoneal metastases (GCPM) but has not been studied with standard-of-care platinum/fluoropyrimidine combinations. Our goal to was evaluate IP-PTX with capecitabine/oxaliplatin (XELOX) in GCPM. METHODS: Forty-four patients with GCPM received IP PTX (40 mg/m2, Days 1, 8), oral capecitabine (1000 mg/m2 twice daily, Days 1-14) and intravenous oxaliplatin (100 mg/m2, Day 1) in 21-day cycles. Patients with synchronous GCPM underwent conversion surgery if they had good response after chemotherapy, conversion to negative cytology, no extraperitoneal metastasis, and no peritoneal disease during surgery. The primary endpoint was overall survival and secondary endpoints were progression-free survival and safety. Outcomes from the trial were compared against a matched cohort of 39 GCPM patients who received systemic chemotherapy (SC) comprising platinum/fluoropyrimidine. RESULTS: The median OS for the IP and SC groups was 14.6 and 10.6 months (hazard ratio [HR] 0.44; 95% confidence interval [CI], 0.26-0.74; p = 0.002). The median PFS for the IP and SC group was 9.5 and 4.4 months respectively (HR 0.39; 95% CI 0.25-0.66; p < 0.001). Patients in the SC group were younger (IP vs. SC, 61 vs. 56 years, p = 0.021) and had better performance status (ECOG 0, IP vs. SC, 47.7% vs. 76.9%, p = 0.007) compared with the IP cohort. In IP group, conversion surgery was performed in 36.1% (13/36) of patients, with a median OS of 24.2 (95% CI 13.1-35.3) months and 1-year OS of 84.6%. CONCLUSIONS: IP PTX with XELOX is a promising treatment option for GCPM patients. In patients with good response, conversion surgery was feasible with favourable outcomes.

Fibrillary Glomerulonephritis and Monoclonal Gammopathy: Potential Diagnostic Challenges.

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease featured by the randomly arranged 12- to 24-nm fibrils under electron microscopy (EM). Up to 10% of FGN patients have monoclonal gammopathy. However, distinguishing between FGN as monoclonal gammopathy of renal significance (MGRS) and FGN from other causes with incidental monoclonal gammopathy of undetermined significance (MGUS) can be challenging, as the current way of demonstrating monoclonality is flawed due to (1) the suboptimal sensitivity of kappa staining by immunofluorescence in frozen tissue (IF-F) as compared to pronase-digested paraffin sections (IF-P), causing incorrect labeling of light chain restriction; (2) the unavailability of immunoglobulin G (IgG) subtyping in some centers; and (3) the unavailability of tests demonstrating the monoclonality of highly variable VH or VL domains in immunoglobulin structures in clinical use. The discovery of DnaJ homolog subfamily B member 9 (DNAJB9) allows diagnosis for FGN with less reliance on EM, and the summary of recent studies revealed that genuine MGRS is extremely rare among FGN. Further research integrating IF-P, IgG subtyping, VH or VL domain monoclonality confirmation, and DNAJB9 as diagnostic modalities, with corresponding clinical data including treatment response and prognosis, is required for a better understanding of this subject.

Sclerosing microcystic adenocarcinoma of the parotid gland - The first recorded case with histo-cytopathologic correlation and a brief review of the literature.

We present a case of a 1.0 cm primary tumor of the left parotid gland that meets the histological criteria for the recently described entity sclerosing microcystic adenocarcinoma. The patient was a 73-year-old man with a concurrent tonsillar squamous cell carcinoma, and a history of nasopharyngeal carcinoma treated with radiotherapy 23 years prior. Fine needle aspiration cytology demonstrated low-grade biphasic basaloid neoplastic cells arranged in branching sheets and clusters with minimal nuclear pleomorphism. A biphasic appearance was apparent and some of the cell clusters were bordered by a layer of flattened cells with ovoid bland nuclei. On histology, the tumor comprised small bilayered infiltrative tubules, nests, cords, and microcysts. On immunohistochemistry, EMA, SOX-10, P63, and S-100 protein highlighted a dual cell population of luminal and abluminal cells. The cells were negative for CD117, and the Ki-67 proliferation index was low (<5%).

Plexiform Fibrohistiocytic Tumor Presenting as a Central Neck Mass Clinically Mimicking a Thyroglossal Duct Cyst: An Unusual Case Reported with Histo-cytopathologic Correlation and a Review of the Cytopathology Literature.

We present the case of an uncommon example of a plexiform fibrohistiocytic tumor (PFHT) occurring in the anterior central neck region of a 40 year-old female with previous subtotal thyroidectomy. The tumor clinically mimics a complicated thyroglossal duct cyst. On fine needle aspiration cytology, the tumor was composed of sheets of bland spindle cells and nests of plump histiocytoid cells in vaguely whorled arrangements. Occasional multinucleated giant cells were also identified. The excised specimen showed an irregular, highly infiltrative subcutaneous tumor arranged in a nodular/plexiform pattern concentrated to the center of the tumor mass. In addition, the tumor contained numerous tongue-like extensions composed of variably cellular, fibroblastic/fibromatosis-like areas. These fibroblastic/fibromatosis-like extensions reached far from the epicenter of the tumor and were associated with scattered small plexiform nodules of histiocytic cells. These tongue-like extensions multifocally involved the surgical margins. The fibroblastic and histiocytoid cells showed diffuse smooth muscle actin (SMA) expression. The multinucleated giant cells and also the histiocytoid proliferation were positive for CD68. This case illustrates an uncommon both anatomical and demographic manifestation of PFHT and also characterize the fine needle aspiration cytologic features in this tumor, which previously have been reported in a few cases.

Primary Pleomorphic Lipoma of the Parotid Gland with Prominent Myxoid Change: Report of a Rare Case Mimicking Carcinoma Ex Pleomorphic Adenoma on Fine Needle Aspiration Cytology.

We present a case of a primary 2.5 cm pleomorphic lipoma of the right parotid gland with prominent myxoid change which on FNA displayed features that mimicked a carcinoma or sarcoma ex pleomorphic adenoma. The patient was a 79 year old man with no oncological history or tumor elsewhere. On immunohistochemistry the neoplastic cells strongly expressed CD34. There was no expression of retinoblastoma protein, smooth muscle actin, S100-protein or cytokeratins (AE1/3 and CAM5.2). The Ki-67 proliferation index was low (< 2%). Fluorescence in situ hybridization was negative for MDM2 gene amplification.

Right adrenal gland pseudocyst masquerading as a large symptomatic hepatic cyst: Single incision laparoscopic (SILS) resection and a review of current literature.

Adrenal pseudocysts are rare entities, which are usually asymptomatic. Large symptomatic adrenal pseudocysts may cause compressive symptoms. The etiology of these cysts is unknown, although the cyst wall is all lined by fibrous tissue, without any epithelial or endothelial lining. We report a case of a 26-year-old lady who presented with a symptomatic right adrenal pseudocyst measuring 7.6 cm in size. Magnetic resonance imaging confirmed the presence of a right retroperitoneal cystic lesion which was hyperintense on T2 sequencing. An attempted single incision transumbilical laparoscopic surgery (SILS) was performed to excise the right adrenal pseudocyst. However, due to the retro-hepatic nature of the lesion and as the medial wall of the cyst was adherent to the inferior vena cava, an additional 5 mm port was inserted to facilitate retraction of the liver. The post-operative period was uneventful. She was successfully discharged from the hospital as a day surgery patient. The final pathology showed an adrenal pseudocyst.

Cabergoline-induced fibrosis of prolactinomas: a neurosurgical perspective.

Presently, the standard of care for prolactinomas, a type of pituitary adenoma, is dopaminergic agents such as bromocriptine and cabergoline. However, dopaminergic agents may induce fibrosis of cardiac valves leading to valvular insufficiency, necessitating surgical treatment of prolactinoma. Fibrosis of prolactinoma can be induced by prolonged medical treatment with bromocriptine, and this usually occurs after years of treatment. In comparison to bromocriptine, there have been no reports of cabergoline-induced fibrosis of prolactinoma. There is a potential for greater emphasis to be placed on assessing the tumour consistency from preoperative MRI scans, or even preoperative contrast-enhanced 3D Fast Imaging Employing Steady-state Acquisition imaging to allow better planning of the surgery. We report a rare case of fibrosis of prolactinoma after cabergoline treatment resulting in its subsequent difficult surgical removal. This patient had early MRI changes of fibrosis of prolactinoma after a short period of 6 months of cabergoline treatment.

Spindle Cell Mucoepidermoid Carcinoma of the Palatine Tonsil With CRTC1-MAML2 Fusion Transcript: Report of a Rare Case in a 17-Year-Old Boy and a Review of the Literature.

Mucoepidermoid carcinoma (MEC) with a predominant spindle cell composition occurring in the palatine tonsil is exceedingly rare. We present a case of a 17-year-old boy with an uncommon spindle cell variant of MEC arising in the palatine tonsil. Histologically, the tumor showed a solid, noncystic architecture and was composed of a predominant population of bland spindle to fusiform cells arranged in organoid nests with interspersed goblet cells and focal areas of ductular structures. Reverse transcriptase polymerase chain reaction and fluorescence in situ hybridization (FISH) revealed the presence of a t(11;19) CRTC1-MAML2 gene fusion in this rare variant of MEC. This is the first case report of a spindle cell MEC of the palatine tonsil, with molecular genetic confirmation. It illustrates the importance of awareness and recognition of this uncommon histological variant of MEC, which will help establish appropriate treatment and prognostication.

Primary pulmonary clear cell sarcoma-the first two reported cases.

We report two cases (male patients 50 and 55 years old) of clear cell sarcoma ("melanoma of soft parts") arising in the lung, of which one case showed regional lymph node metastases. Histologically, both tumors displayed varying clear epithelioid and spindle neoplastic cells arranged in storiform and nested growth patterns, separated by thin fibrovascular septa. Immunohistochemical studies demonstrated positive expression of S-100 protein, HMB-45 and Melan-A in one case and S-100 protein only in the other. Fluorescence in situ hybridization showed positive EWSR1 gene rearrangement, and a presence of EWS-ATF1 fusion transcript was confirmed by RT-PCR and sequencing in one case.

Maxillary mesenchymal chondrosarcoma presenting with epistaxis in a child.

Mesenchymal chondrosarcomas are a rare variant of primary chondrosarcomas and can pose a diagnostic dilemma, especially when the features on conventional imaging are equivocal for an aggressive lesion. There is very little PET-CT experience in mesenchymal chondrosarcomas as per the literature and to the best of our knowledge, we are the first to describe a maxillary mesenchymal chondrosarcoma on PET-CT imaging. We report a case where PET-CT not only complemented conventional imaging in suspecting a malignant osseous lesion, but also was indicative of the grade of the tumor.

Carcinoma cuniculatum of the esophagus and tongue: report of two cases, including TP53 mutational analysis.

Carcinoma cuniculatum (CC) is a rare variant of extremely well differentiated squamous cell carcinoma. We present the clinicopathological features of two cases of CC; one lingual and one esophageal case with a molecular genetic study regarding the TP53 gene mutational status. Case 1 was a 62 year old male with enlarging chronic ulcer in the tongue. Case 2 was a 77 year old male with progressive dysphagia and odynophagia. Both patients were treated surgically. Both tumors showed deeply invaginating, keratin-filled, burrowing crypts lined by very well differentiated squamous epithelium. The esophageal tumor showed varying degrees of reactive nuclear atypia largely limited to the areas with dense intratumoral infiltration of neutrophils. No mutation of TP53 was identified in the esophageal case. Cytologic atypia limited to areas of significant acute inflammation may occur in CC and should, in the absence of aggressive stromal invasion, not preclude a diagnosis of CC.