Recurrent peritoneal carcinomatosis in the scrotum in a patient with a history of mucocele of the appendix.

Paratesticular metastases are rare entities and a paratesticular metastasis of a mucinous appendiceal adenocarcinoma with peritoneal carcinomatosis has not been reported in the literature in the last 15 years. We present a case of mucinous appendiceal adenocarcinoma that progressed to peritoneal carcinomatosis. Twenty months following surgery and intraperitoneal therapy, the patient presented with testicular pain and swelling. Ultrasound examination raised suspicion for a neoplastic process and pathology confirmed a metastatic mucinous adenocarcinoma of appendiceal origin. Radiologists should be cognizant of potential paratesticular metastases in patients with a known history of malignancy with new testicular pain and swelling.

Increased signal of the fibular collateral ligament of the knee on MRI, clinically significant?

INTRODUCTION: The purpose of this study was to determine the clinical significance of signal hyperintensity in the proximal fibular collateral ligament (FCL) on coronal proton density (PD) fat-saturated (FS) MRI of the knee, a common finding. This study is unique in that it characterizes the FCL of a comprehensive, large cohort of both symptomatic and asymptomatic patients, which to our knowledge represents the first study with such broad inclusion criteria. METHODS: A large case series was performed analyzing MRI of the knee of 250 patients from July 2021 through September 2021 and retrospectively reviewed. All studies were performed on 3-Tesla MRI scanners with a dedicated knee coil and in accordance with standard institutional knee MRI protocol. Signal in the proximal fibular collateral ligament was assessed on coronal PDFS and axial T2-weighted FS images. Increased signal was classified as none, mild, moderate, or severe. A corresponding chart review of clinic notes was performed to determine the presence or absence of lateral knee pain. An FCL sprain or injury was considered present if the medical record described tenderness on palpation of the lateral knee, positive finding against resistance to the leg (varus stress test) or reverse pivot shift, or any clinical suspicion for lateral complex sprain or posterolateral corner injury. RESULTS: The majority (74%) of knee MRIs demonstrated the presence of increased signal in the proximal fibular collateral ligament on coronal PD FS images. <5% of these patients had associated clinical findings of fibular collateral ligament and/or lateral supporting structure injury. DISCUSSION: Although increased signal in the proximal FCL of the knee is a common finding on coronal PDFS images, the majority are not associated with clinical symptoms. Thus, this increased signal is likely not a pathological finding in the absence of clinical findings of fibular collateral ligament sprain/injury. Our study emphasizes the importance of clinical correlation in identifying increased signal in the proximal FCL as pathologic.

Imaging findings in pediatric type 1 Gaucher disease: what the clinician needs to know.

This study presents visceral and skeletal imaging findings commonly observed in pediatric patients with type I Gaucher disease. Presented images show methods used for radiologic assessment of pediatric Gaucher patients, and imaging findings are discussed in the context of the underlying pathophysiology of the disease. Routine radiologic surveillance plays a central role in assessing Gaucher disease progression and response to treatment, but monitoring of pediatric patients presents specific challenges with regard to minimizing radiation exposure and interpreting extent of marrow involvement against the backdrop of normal growth-related changes in marrow composition. In addition to highlighting imaging findings in children with type I Gaucher disease, this manuscript discusses alternate modalities, which minimize radiation and may be just as accurate, if not better, than conventional methods exposing the child to radiation.

Isopentenyl pyrophosphate-activated CD56+ {gamma}{delta} T lymphocytes display potent antitumor activity toward human squamous cell carcinoma.

PURPOSE: The expression of CD56, a natural killer cell-associated molecule, on alphabeta T lymphocytes correlates with their increased antitumor effector function. CD56 is also expressed on a subset of gammadelta T cells. However, antitumor effector functions of CD56(+) gammadelta T cells are poorly characterized. EXPERIMENTAL DESIGN: To investigate the potential effector role of CD56(+) gammadelta T cells in tumor killing, we used isopentenyl pyrophosphate and interleukin-2-expanded gammadelta T cells from peripheral blood mononuclear cells of healthy donors. RESULTS: Thirty to 70% of expanded gammadelta T cells express CD56 on their surface. Interestingly, although both CD56(+) and CD56(-) gammadelta T cells express comparable levels of receptors involved in the regulation of gammadelta T-cell cytotoxicity (e.g., NKG2D and CD94), only CD56(+) gammadelta T lymphocytes are capable of killing squamous cell carcinoma and other solid tumor cell lines. This effect is likely mediated by the enhanced release of cytolytic granules because CD56(+) gammadelta T lymphocytes expressed higher levels of CD107a compared with CD56(-) controls following exposure to tumor cell lines. Lysis of tumor cell lines is blocked by concanamycin A and a combination of anti-gammadelta T-cell receptor + anti-NKG2D monoclonal antibody, suggesting that the lytic activity of CD56(+) gammadelta T cells involves the perforin-granzyme pathway and is mainly gammadelta T-cell receptor/NKG2D dependent. Importantly, CD56-expressing gammadelta T lymphocytes are resistant to Fas ligand and chemically induced apoptosis. CONCLUSIONS: Our data indicate that CD56(+) gammadelta T cells are potent antitumor effectors capable of killing squamous cell carcinoma and may play an important therapeutic role in patients with head and neck cancer and other malignancies.

Adenomyomatous hyperplasia of the gallbladder with perineural invasion: revisited.

We report 9 examples of segmental adenomyomatous hyperplasia of the gallbladder with perineural invasion. Five patients were women and 4 men. Their ages ranged from 49 to 81 years (mean age 64 y). Eight patients had gallbladder calculi. The original pathologic diagnosis of adenocarcinoma was made in 5 patients and of "adenoma malignum" in one. Six patients are disease-free for 2 to 11 years following cholecystectomy, 1 patient died of unrelated causes and 2 were lost to follow-up. Histologically 2 types of adenomyomatous hyperplasia were recognized. The first one characterized by numerous Rokitansky-Aschoff sinuses (RASs) was accompanied by smooth muscle hyperplasia and an expanded subserosal layer containing numerous nerve trunks (6 cases). The second type was characterized by an extensively fibrotic gallbladder wall with numerous RASs but with few or no smooth muscle bundles and an expanded subserosal layer containing abundant nerve-trunks (3 cases). Perineural (7 cases) and intraneural invasion (2 cases) was identified only in the subserosal layer. The lack of p53 reactivity and the very low MIB-1-labeling index provide additional support to the non-neoplastic nature of the lesion. The pseudoinvasive pattern of the RASs, reactive epithelial atypia, and the perineural and intraneural invasion probably contributed to the erroneous diagnosis of adenocarcinoma or "adenoma malignum." The mechanism by which the epithelial structures "invaded" the perineural spaces and the nerves is unclear. We favor the hypothesis that the migration of the benign glandlike structures into the nerves is related to the production of chemotactic factors or signaling substances and the activation of cell receptors.

Paranasal sinus osteomas and Gardner's syndrome.

OBJECTIVES: Osteomas are common benign tumors of the paranasal sinuses. The origin of these lesions is uncertain. Although most are asymptomatic, symptoms can include headaches, facial pain, rhinorrhea, and sinusitis. Osteomas are also seen as part of Gardner's syndrome, an autosomal dominant disease characterized by intestinal polyposis, osteomas, and cutaneous and soft tissue tumors. In affected individuals, the risk of developing colon cancer approaches 100%. On average, osteomas are detected 17 years before colon polyps appear. METHODS: Three patients with maxillary or ethmoid osteomas and chronic sinusitis are presented. RESULTS: One of the patients had evidence of Gardner's syndrome, based on the presence of gastrointestinal symptoms and a positive family history of polyposis. CONCLUSIONS: Otolaryngologists should be aware of the possibility of Gardner's syndrome in patients with paranasal sinus osteomas. Suspected patients should have a complete workup for Gardner's syndrome, including lower gastrointestinal tract endoscopy, barium enema imaging, and DNA testing.

Comparison of performance characteristics between new and reprocessed electrophysiology catheters.

OBJECTIVE: Electrophysiology (EP) catheter reprocessing is widespread and previous studies have examined clinical performance, sterility and safety of these reprocessed devices. Here we compare the intrinsic engineering characteristics, electrical, mechanical and safety, between new and reprocessed devices. MATERIALS AND METHODS: New (58 devices) and clinically used (five times)/reprocessed (165 devices) EP catheters from five manufacturers were employed to examine and compare catheter electrode continuity, electrode isolation, electrical leakage current, shaft torsion and stiffness characteristics as well as tip buckling and bond strengths. RESULTS: Electrode continuity, isolation and leakage currents for both new and reprocessed EP catheters were within acceptance criteria for electrode continuity (<30 Omega) and shaft electrical leakage (<0.5 mA) as well as electrode isolation (>200 Omega). In addition, reprocessed catheters were equivalent when compared with their new counterparts. While catheter shaft torque forces varied five fold amongst manufacturers, comparison between new and reprocessed devices within a manufacturer showed no significant differences. Likewise shaft stiffness showed no significant difference between new and reprocessed devices. Average tip buckling forces for all catheters were substantially below the acceptance criteria of 0.45 lb (between 0.04 and 0.1 lb) with differences between new and reprocessed catheters not being significant. All bond strengths for both new and reprocessed catheters exceeded the acceptance criteria specified in ISO 10555-1. CONCLUSIONS: This study found that reprocessed EP catheters which had undergone five actual clinical use/reprocessing cycles met and exceeded acceptance criteria specified by industry standards as well as individual manufacturer's criteria for both electrical and safety characteristics. We conclude that reprocessed EP catheters exhibit electrical, mechanical and safety characteristics which are equivalent to their new counterparts.

Angiofollicular lymphoid hyperplasia (Castleman disease) presenting as an isolated neck mass.

Castleman disease is a relatively uncommon lymphoproliferative disorder. A presentation as an isolated lesion in the neck is extremely rare. We report the case of a 26-year-old man who presented with a 4-month history of an enlarging right neck mass that was identified as Castleman disease on excisional biopsy. The biopsy was curative. In localized forms of Castleman disease, excisional biopsy is both diagnostic and curative. However, in multicentric forms, additional treatment is required.

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS): an indication for tonsillectomy.

Children with obsessive compulsive disorder or tic disorders that are associated with streptococcal infections (Group A beta-hemolytic) in the oro-pharyngeal region are given the diagnosis of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). Tonsillectomy has been reported to resolve the neuro-psychiatric symptoms in these children. We have a case of a 9-year-old boy who was seen in our clinic with multiple recurrent streptococcal infections of the oro-pharyngeal cavity. He also exhibited neuro-psychiatric symptoms including agitation, hyperactivity, and tics. These symptoms followed his recurrent infections. Tonsillectomy was performed and in one year follow-up the patient did not have any recurrent streptococcal infections, and his neuro-psychiatric symptoms resolved completely. Guidelines for medical and surgical management of recurrent strep infections in the face of PANDAS are reviewed.

Rare combination of frontonasal and bilateral naso-orbital encephaloceles.

Encephaloceles, while a common entity affecting 1:4000 live births, typically occur in the occipital region. Encephaloceles involving the frontal region comprise only 15% of all cases. Naso-orbital encephaloceles are rarely seen. Our case profiles a child born at term with an atrial septal defect (ASD), micrognathia, cleft lip, and frontonasal as well as bilateral naso-orbital encephaloceles. At birth the encephaloceles were undetected. During the cleft palate pre-operative preparation, the bilateral naso-orbital encephaloceles were diagnosed as dacrocystoceles for which the child underwent surgical repair. Misdiagnosis and loss to follow up lead to delayed surgical treatment until the child was almost two years of age; the right eye was near complete closure due to the increasing size of the encephalocele. This case highlights the importance of meticulous radiologic interpretation of midline nasal masses, as a correct diagnosis impacts clinical management and directs surgical repair.

Ectopic nasopharyngeal pituitary adenoma resected with endoscopic technique.

We describe the case of an 80-year-old man with diabetes who presented with nonspecific dizziness. He was found on magnetic resonance imaging to have a 3.2-cm mass within the posterior and central aspect of the nasopharynx. Nasal endoscopy showed that the mass, which arose from the posterior edge of the nasal septum and was attached to the superior nasopharynx, was narrowing the patient's airway. Although initially the mass was suspected to be a minor salivary gland tumor, histopathologic analysis led to a diagnosis of pituitary adenoma. The ectopic tumor was removed via transnasal en bloc resection with partial adjacent septal resection. Final pathology confirmed the diagnosis. The patient had an uneventful recovery and no sequellae at 2-year follow-up.

Microduplication 22q11.2, an emerging syndrome: clinical, cytogenetic, and molecular analysis of thirteen patients.

Chromosome 22, particularly band 22q11.2, is predisposed to rearrangements due to misalignments of low-copy repeats (LCRs). DiGeorge/velocardiofacial syndrome (DG/VCFS) is a common disorder resulting from microdeletion within the same band. Although both deletion and duplication are expected to occur in equal proportions as reciprocal events caused by LCR-mediated rearrangements, very few microduplications have been identified. We have identified 13 cases of microduplication 22q11.2, primarily by interphase fluorescence in situ hybridization (FISH). The size of the duplications, determined by FISH probes from bacterial artificial chromosomes and P(1) artificial chromosomes, range from 3-4 Mb to 6 Mb, and the exchange points seem to involve an LCR. Molecular analysis based on 15 short tandem repeats confirmed the size of the duplications and indicated that at least 1 of 15 loci has three alleles present. The patients' phenotypes ranged from mild to severe, sharing a tendency for velopharyngeal insufficiency with DG/VCFS but having other distinctive characteristics, as well. Although the present series of patients was ascertained because of some overlapping features with DG/VCF syndromes, the microduplication of 22q11.2 appears to be a new syndrome.