Partial Gastric Resection for Symptomatic Anemia following Diagnosis of Merkel Cell Carcinoma (MCC) of the Skin with Gastric Metastasis.

Merkel Cell Carcinoma (MCC) is a rare dermatologic malignancy with significant morbidity and mortality associated with metastatic disease. In this case, we discuss and extremely rare presentation of MCC with metastasis to the stomach in a patient that presented with profound anemia. Unfortunately, mortality following diagnosis of MCC with gastric metastasis approaches 67% at 4 months based on available published reports. Due to its rarity and high rate of mortality, there is a lack of available research and literature to help guide treatment of this rare presentation of MCC. This case report presents a positive outcome associated with a partial gastrectomy for the treatment of symptomatic MCC with gastric metastasis and continued survival with persistently stable hemoglobin at 12 months. Key clinical message: Anemia may be a significant cause of the morbidity and mortality associated with MCC with gastric metastasis. Our case demonstrates a positive outcome associated with partial gastric resection and presents a possible treatment option for this rare disease process.

Primary Cutaneous B-Cell Lymphoma: Management and Patterns of Recurrence at the Multimodality Cutaneous Lymphoma Clinic of The Ohio State University.

BACKGROUND: The increasing incidence of primary cutaneous B-cell lymphomas (PCBCLs) presents new challenges for clinicians. Despite advances in the clinical and pathologic characterization of PCBCL, the significance of the current staging approach as a risk profiling tool and the effect of various treatments on outcome remain unclear. MATERIALS AND METHODS: We retrospectively reviewed patients who presented with a diagnosis of PCBCL seen at The Ohio State University between 1998 and 2012. We reviewed the initial presentation and treatment modality. We then assessed whether the treatment modality (conservative skin-directed vs. definitive radiation with or without systemic therapy), stage (T1 or ≥T2), or histologic subtype (primary cutaneous follicle center lymphoma [PCFCL] vs. primary cutaneous marginal zone B-cell lymphoma [PCMZL]) affected the risk of recurrence. RESULTS: We identified 67 patients referred with an initial diagnosis of PCBCL. After imaging, 12 did not meet the criteria for PCBCL and were classified as having systemic B-cell lymphoma with cutaneous involvement. The remaining 55 patients included 25 with PCMZL, 24 with PCFCL, 2 with primary cutaneous large B-cell lymphoma leg type, and 4 with unclassifiable disease. According to the International Society of Cutaneous Lymphoma-European Organization for Research and Treatment of Cancer staging, 30 cases were T1 (55%), 14 T2 (25%), and 11 T3 (20%). Comparing the time to first recurrence (TFR) by indolent PCBCL subtypes, we found no difference in the recurrence risk for either stage (T1, p = .51 vs. T2/T3, p = .30). Comparing TFR by treatment modality, we found no difference in TFR within T1 patients (p = .34) or T2/T3 patients (p = .44). CONCLUSION: Our limited analysis highlights the importance of complete staging at diagnosis and suggests that the treatment modality does not affect the risk of recurrence in T1 indolent PCBCL.

Comprehensive evaluation of caspase-14 in vulvar neoplasia: an opportunity for treatment with black raspberry extract.

OBJECTIVE: The aim of this study is to determine the expression of caspase-14, a key protein in maturation of squamous epithelia, in archival malignant and premalignant vulvar squamous lesions and examine in-vitro effects of a black raspberry extract (BRB-E) on a vulvar squamous cell carcinoma (VSCC) cell line. METHODS: VSCC cell cultures were exposed to different BRB-E concentrations and used to create cell blocks. Immunohistochemistry for caspase-14 was performed on cell block sections, whole tissue sections, and a tissue microarray consisting of normal vulvar skin, lichen sclerosus (LS), classic and differentiated vulvar intraepithelial neoplasia (cVIN and dVIN respectively), and VSCC. RESULTS: LS demonstrated abnormal full thickness (5/11) or absent (1/11) caspase-14 staining. dVIN often showed markedly reduced expression (4/7), and cVIN occasionally demonstrated either absent or reduced caspase-14 (6/22). VSCC predominantly had absent or markedly reduced caspase-14 (26/28). VSCC cell cultures demonstrated a significant increase in caspase-14 (p=0.013) after BRB-E treatment: 7.3% (±2.0%) of untreated cells showed caspase-14 positivity, while 21.3% (±8.9%), 21.7% (±4.8%), and 22.6% (±5.3%) of cells were positive for caspase-14 after treatment with 200, 400, and 800 µg/mL BRB-E, respectively. Pair-wise comparisons between the treatment groups and the control demonstrated significant differences between no treatment with BRB-E and each of these treatment concentrations (Dunnett's adjusted p-values: 0.024, 0.021, and 0.014, respectively). CONCLUSIONS: Caspase-14 is frequently decreased in premalignant and malignant vulvar squamous lesions, and is upregulated in VSCC cell culture by BRB-E. BRB-E should be further explored and may ultimately be incorporated in topical preparations.

Squamous cells in effusions: A study of 24 cases.

INTRODUCTION: Malignant squamous cells in serous effusions are rare. We present our experience with squamous cell carcinoma (SqCC) and benign squamous cells in effusions. MATERIALS AND METHODS: Specimens were retrieved from our database using search codes as "squamous" within the final diagnosis under various serous effusions. RESULTS: Twenty-nine specimens were recovered, and 5 of those were excluded. Of the 24 specimens, 3 were duplicates; therefore, only the first specimen from each was included for a final tally of 21 specimens. Specimens were from pleural fluid (n = 16, 76%), pericardial fluid (n = 2), pelvic fluid (n = 2), and peritoneal fluid (n = 1). Nineteen were SqCC (primary sites: 7 lung, 2 uterine cervix, 2 larynx, 2 anus, 2 esophagus, 1 tongue, 1 mandible, 1 skin, 1 vulva), and 2 patients had benign squamous cells only (1 from a ruptured esophageal adenocarcinoma and 1 from a nonmalignant esophageal rupture). In SqCC cases, a round-oval cell with dense cytoplasm was the predominant cell type (n = 12) followed by undifferentiated cells (n = 4), polygonal-type cells (n = 2), and fiber-type cells (n = 1). Of the SqCC specimens, 12 (63%) showed varying degrees of keratinization. Other features in SqCC cases included refractile rings (89%), keratin pearls (53%), and vacuolated cytoplasm (42%). Herxheimer spirals were absent. Two benign cases showed polygonal cell morphology only. All patients with SqCC died shortly after fluid collection (range 2-313 days; mean: 58.1 days). CONCLUSIONS: Metastatic SqCC in serous effusions are rare, primarily arranged as single cells with rounded nuclei lacking visible nucleoli surrounded by a minimal amount of dense cytoplasm, and represent a dismal prognosis.

Hybrid capture 2 test results after an initial equivocal RLU/CO value are dependent on age.

The effect of age on Hybrid Capture 2 (HC2) tests initially falling within the equivocal range has not been determined. We identified 359 cervicovaginal liquid cytology specimens with initial equivocal values. First and second retest relative light units/cutoff (RLU/CO) values were compared for women of 3 different age groups (15-29, 30-49, and ≥50 years). The proportion of first retests with an RLU/CO of less than 1 increased with age (P < .001). Of the 56 second retests performed, only 4 had an RLU/CO of 1 or more. The proportion of "positive" HC2 results following the current HC2 algorithm decreased with increasing age (P < .001), showing that HC2 test results after an initial equivocal value are dependent on age. Follow-up demonstrated cervical intraepithelial neoplasia grade 2 or worse (CIN2+) in 6 (5.9%) women 15 to 29 years old and in 5 (6.3%) women 30 to 49 years old. No CIN2+ was found on follow-up of 34 of 57 women 50 years and older. These results likely reflect human papillomavirus infection prevalence and question the use of identical cutoff values regardless of age for detection of CIN2+.

Amyloidomas of soft parts: diagnosis by fine-needle aspiration.

A tumoral mass of amyloid (amyloidoma) arising in the soft tissues of the distal extremities is exceedingly unusual, and applying the fine-needle aspiration (FNA) biopsy technique to specifically diagnose this condition is even more infrequent. Herein, we report an example of two separate amyloidomas arising in the right calf of an otherwise asymptomatic elderly man who was referred to the sarcoma clinic because these masses clinically and radiographically simulated a malignant neoplasm. The fine-needle aspirate yielded amorphous, acellular, dense blue material on Romanowsky-stained smears and smudgy acellular cyanophilic material on Papanicolaou-stained smears. The aspirate procured cell-block contained hyalinized eosinophilic acellular material on H&E stain that was Congo-red positive and displayed yellow-green birefringence with polarization. Unlike prior assertions to the contrary, a diagnosis of amyloidoma is possible using FNA cytopathology even in patients without a known underlying amyloidogenic disorder.

Sclerosing epithelioid fibrosarcoma of the oral cavity.

Sclerosing epithelioid fibrosarcoma (SEF) rarely occurs outside the somatic soft tissue. Until recently no consistently specific genetic alteration had been associated with SEF. Molecular testing of the FUS gene rearrangement involving chromosome 16 [at one time considered specific for low-grade fibromyxoid sarcoma (LGFMS) and its variant, LGFMS with giant collagen rosettes), may be a nonrandom abnormality in some cases of SEF.We present an example of a rare FUS-positive SEF that arose in the floor of mouth of a 56 year old male. Light microscopy, exhaustive immunohistology, and FISH examination showing chromosome rearrangement using the FUS break-apart probe led to an erroneous diagnosis of LGFMS with giant collagen rosettes. An outside expert agreed with that diagnosis citing the FISH results as confirmatory. Upon review almost 2 years later after local recurrence, the classic histopathologic features of SEF were noted instead. This example suggests that at least a subset if not most examples of SEF are part of the LGFMS "family" of neoplasms, and reiterates the value of careful histologic examination in an age of increasingly sophisticated and presumably specific molecular results.

Cytopathology of "double-hit" non-Hodgkin lymphoma.

BACKGROUND: B-cell lymphomas with concurrent IGH-BCL2 and c-MYC rearrangements (so-called "double-hit lymphomas" [DHL]) are a relatively rare, recently described category in the 2008 World Health Organization classification of hematopoietic neoplasms. Response to chemotherapy and survival are poor. METHODS: The authors reviewed files of cytogenetically documented DHL to identify cytologic features that would allow its possible recognition. RESULTS: Twelve fine-needle aspirates (FNAs), 2 pleural fluids, and 1 touch imprint of cytogenetically proven DHL were uncovered. Primary DHL was correctly recognized in 3 of 12 FNA cases using Ki-67 staining coupled with a positive bcl-2 result as the basis for performing fluorescence in situ hybridization (FISH) analysis of c-MYC and IGH-BCL2 rearrangements. Remaining FNAs and non-FNA cases were diagnosed as non-Hodgkin lymphoma, B-cell lymphoma, or atypical lymphocytosis. Ten cases had cell block material available. All cases had high cellularity with a dissociated smear pattern and background lymphoglandular bodies. Cell size ranged from intermediate to large. Nuclei were predominantly rounded or slightly irregular in contour; 4 FNAs had markedly cleaved nuclei. Some nuclei harbored discrete but small nucleoli, whereas in others coarse chromatin and indistinct or multiple small nucleoli existed. A variable number of mitotic figures, tingible body macrophages, and background apoptotic cells were also present. CONCLUSIONS: No specific cytomorphologic feature(s) were found to reliably identify DHL using FNA or exfoliative cytology. A high Ki-67 proliferation index and positive bcl-2 staining (on cytospin slides or cell block material) of cases not conforming to typical Burkitt lymphoma morphology should prompt FISH analysis for c-MYC and/or IGH-BCL2 rearrangements to identify DHL, particularly if tissue biopsy is not expected.

Pseudo-outbreak of Cupriavidus pauculus infection at an outpatient clinic related to rinsing culturette swabs in tap water.

Cupriavidus pauculus is a water microorganism rarely isolated from clinical specimens. We describe a pseudo-outbreak in which multiple strains that were associated with moistening of culturette swabs with tap water were isolated from a single clinic before collecting the patient specimen.