CD161 Is Expressed in a Subset of T-Cell Prolymphocytic Leukemia Cases and Is Useful for Disease Follow-up.

OBJECTIVES: CD161 (NKRP1) is a lectin-like receptor present on NK cells and rare T-cell subsets. We have observed CD161 expression in some cases of T-cell prolymphocytic leukemia (T-PLL) and found it to be useful in follow-up and detection of disease after treatment. METHODS: Retrospective review of T-PLL cases with complete flow cytometry data including CD161. RESULTS: We identified 10 cases of T-PLL with flow cytometric evaluation of CD161 available. Six of these cases were positive for CD161 expression. All CD161-positive cases were positive for CD8 with variable CD4 expression, whereas all CD161-negative cases were negative for CD8. In a case with two neoplastic subsets positive and negative for CD8, only the former expressed CD161. CONCLUSIONS: These novel results suggest that CD161 is often aberrantly expressed in a defined subset of T-PLL positive for CD8. We are showing the utility of this immunophenotype in diagnosis and follow-up.

Development of cutaneous squamous cell carcinoma after prolonged exposure to pegylated liposomal doxorubicin and hand-foot syndrome: a newly recognized toxicity.

Pegylated liposomal doxorubicin (PLD) can be administered for prolonged periods with minimal toxicity. The risk of cutaneous squamous cell carcinoma (SCC) with this therapy has not been reported. We describe cutaneous SCC of the plantar foot in two patients exposed to high doses of PLD. A 50-year-old man with angiosarcoma received a total PLD dose of 1350 mg/m2 and developed cutaneous SCC of bilateral plantar feet. A 45-year-old woman with cutaneous T-cell lymphoma was treated with a total PLD dose of 1142 mg/m2 with subsequent diagnosis of cutaneous SCC of the right plantar foot. No risk factors for SCC of the plantar foot were identified in either patient. Cutaneous SCC is likely an unreported side effect of prolonged exposure to PLD. An extended duration of hand-foot syndrome from other anti-cancer drugs may also share this risk. Regular complete skin examination with early intervention for suspicious lesions is indicated in this patient population.

Colorectal Lymphoma: A Contemporary Case Series.

BACKGROUND: Colon and rectal lymphomas are rare and can occur in the context of posttransplant lymphoproliferative disorder. Evidence-based management guidelines are lacking. OBJECTIVE: The purpose of this study was to characterize the presentation, diagnosis, and management of colorectal lymphoma and to identify differences within the transplant population. DESIGN: This was a retrospective review of patients evaluated for colorectal lymphoma between 2000 and 2017. Patients were identified through clinical note queries. SETTINGS: Four hospitals within a single health system were included. PATIENTS: Fifty-two patients (64% men; mean age = 64 y; range, 26-91 y) were identified. No patient had <3 months of follow-up. Eight patients (15%) had posttransplant lymphoproliferative disorder. MAIN OUTCOME MEASURES: Overall survival, recurrence, and complications in treatment pathway were measured. RESULTS: Most common presentations were rectal bleeding (27%), abdominal pain (23%), and diarrhea (23%). The most common location was the cecum (62%). Most frequent histologies were diffuse large B-cell lymphoma (48%) and mantle cell lymphoma (25%). Posttransplant lymphoproliferative disorder occurred in the cecum (n = 4) and rectum (n = 4). Twenty patients (38%) were managed with chemotherapy; 25 patients (48%) underwent primary resection. Mass lesions had a higher risk of urgent surgical resection (35% vs 8%; p = 0.017). Three patients (15%) treated with chemotherapy presented with perforation requiring emergency surgery. Overall survival was 77 months (range, 25-180 mo). Patients with cecal involvement had longer overall survival (96 vs 26 mo; p = 0.038); immunosuppressed patients had shorter survival (16 vs 96 mo; p = 0.006). Survival in patients treated with surgical management versus chemotherapy was similar (67 vs 105 mo; p = 0.62). LIMITATIONS: This was a retrospective chart review, with data limited by the contents of the medical chart. This was a small sample size. CONCLUSIONS: Colorectal lymphoma is rare, with variable treatment approaches. Patients with noncecal involvement and chronic immunosuppression had worse overall survival. Patients with mass lesions, particularly cecal masses, are at higher risk to require urgent intervention, and primary resection should be considered. See Video Abstract at http://links.lww.com/DCR/A929.

Lymph Node With Extensive Involvement by Cryptococcus Shortly Following Liver Transplantation.

Herein, we present a case of extensive lymph node involvement by disseminated Cryptococcus infection developing in the immediate period after liver transplantation and initiation of immunosuppressive therapy. The patient, a 56 year old ethnicity unknown man, received a liver transplant for acute decompensated liver. Beginning 24 days after transplantation, he was found to have Cryptococcus neoformans infection, involving the pleural fluid, blood, cerebrospinal fluid (CSF), liver, and lymph nodes. He received treatment with amphotericin B and flucytosine; he was transitioned to fluconazole, and his response was good. This relatively rapid development of disease raises the possibility of donor-derived Cryptococcus infection.

Buccal epithelial cells display somatic, bone marrow-derived CALR mutation.

Buccal epithelial cells harbor an MPN-associated CALR mutation in a patient with CALR-mutant essential thrombocytosis, Ph+ CML, and no germ line CALR mutation.

Comparative immunomorphology of testicular Sertoli and sertoliform tumors.

Sertoli cell (SC) and sertoliform tumors of the testis are very uncommon; for this reason their differential diagnosis and classification can be challenging. We applied an extensive immunophenotypic panel that included androgenic hormones, enzymes and receptors, neuroendocrine, lineage and genitourinary markers to a series of these lesions to determine if and which immunostains can aid in their diagnostic workup. Study cases included: 2 androgen insensitivity syndrome-associated SC adenomas, 3 SC tumors (SCT) not otherwise specified (SCT-NOS), 3 sclerosing SCT, 2 large cell calcifying SCT, 1 SCT with heterologous sarcomatous elements, 1 malignant SCT, and 1 sertoliform rete testis adenoma (sertoliform RTA). We found that SCT-NOS and variants with sclerosis showed a phenotype akin to atrophic seminiferous tubules characterized by gain of expression of pankeratin, calretinin, CD56, which are negative in normal SC. Distinctive phenotypes were identified in: sclerosing SCT: androgen receptors (AR) + (strong)/PAX2/PAX8+ (subset)/S100+/inhibin-; large cell calcifying SCT: calretinin+ (strong)/S100+/AR-; sertoliform RTA: PAX2/PAX8+/pankeratin+/inhibin-. Androgenic hormones and enzymes did not show diagnostic utility. A panel of calretinin, inhibin, pankeratin, S100, PAX2/PAX8, and AR consistently allowed distinction between variants of Sertoli and sertoliform tumors.

Aggressive angiomyxoma: case report and review of the literature.

A 47-year-old female presented to clinic with a 5-year history of a left buttock mass. The patient's hemoglobin was low (9.7 g/dL); laboratory analysis was otherwise unremarkable. Ultrasound of the left gluteal region demonstrated a heterogeneous vascular solid lesion. Magnetic resonance and computed tomography imaging showed an enhancing mass extending from the left ischioanal fossa through the levator ani muscle into the pelvis. Biopsy revealed bland-appearing spindle-shaped cells positive for estrogen and progesterone receptors, consistent with an aggressive angiomyxoma. The mass was surgically excised without complication. To date, follow-up imaging has not demonstrated evidence of tumor recurrence.

Immunophenotypic differences between neoplastic and non-neoplastic androgen-producing cells containing and lacking Reinke crystals.

We performed a detailed morphologic, immunophenotypic, and endocrine characterization of neoplastic and non-neoplastic lesions of androgen-producing cells known to harbor or lack Reinke crystals (RCs) with an aim to provide further insight into the nature of these cells and crystals. Study cases were selected from the files of participating hospitals and subclassified according to current classifications: 20 with Leydig cell tumors (LCTs), 2 with testicular adrenal rest tumors (TARTs), 2 with testicular tumors of adrenogenital syndrome (TTAGS), and 2 with androgen insensitivity syndrome (AIS). An extensive immunophenotypic panel including markers used in sex cord-stromal cell tumors, androgen hormones, enzymes, and receptors was applied to the cases and 10 non-tumoral adrenal glands. Non-tumoral tissues were scored separately. RCs were present in 90 % of LCT cases and all cases with normal Leydig cells; RCs stained specifically with calretinin and 3ß-hydroxysteroid dehydrogenase (3BHSD) and were present only in cells with high concomitant expression of both proteins, a phenotype unique to Leydig cells and LCTs. Leydig cells from AIS cases lack RCs due to decreased expression of 3BHSD. Calretinin is decreased in testicular adrenal-like tumors and absent in normal adrenocortical cells, which explain why they lack RCs. Calretinin expression in androgen-producing cells is independent from androgen receptors and androgen synthesis. RCs represent for the most part, if not exclusively, crystallized forms of a 3BHSD/calretinin complex. Androgen-producing cells containing and lacking RCs differ mainly in the level of expression of these proteins and androgen receptors.

Undifferentiated (anaplastic) carcinoma with osteoclast-like giant cells of the pancreas: a series of 5 cases with clinicopathologic correlation and cytomorphologic characterization.

INTRODUCTION: Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas (UCOGCP) is a rare neoplasm involving the pancreas. Although typically diagnosed initially via endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), cytomorphologic characterization of the neoplasm has been limited to individual cases in the literature. MATERIALS AND METHODS: Five cases were identified in a retrospective review of our institution's records from 2006 to 2015. Cytomorphologic, immunophenotypic, and corresponding clinical features of the neoplasm are examined and described. RESULTS: UCOGCP accounted for 0.9% of all new pancreatic neoplastic diagnoses, had a median greatest dimension of 4.3 cm, were variably located within the pancreas, and had variable features by radiologic imaging. Patients were of a median age of 78 years old at diagnosis, and had a median length of survival of 10 months. Smear-based cytomorphology and histomorphology from cell block preparations show atypical/pleomorphic mononuclear carcinomatous and bland osteoclast-like giant cellular populations. The immunophenotype of the mononuclear carcinomatous component was CD68, CD99, CK7 (variably), CKAE1/AE3 (variably), and, rarely, p40-positive. The osteoclast-like giant cells positively expressed CD68 and CD99. CONCLUSIONS: Initial diagnosis of UCOGCP is frequently made via EUS-FNA of pancreas tumors, with cytomorphologic features on smears and hematoxylin and eosin stained slides prepared from cell block material being characteristic for the diagnosis. Although the cellular constituents have a consistent immunophenotype, the diagnosis can be based on the morphologic features alone. UCOGCP is an important diagnosis as it may have a distinct clinical course from undifferentiated carcinomas of the pancreas lacking osteoclast-like giant cells.

The mystery of the vanishing Reinke crystals.

Reinke crystals (RC) are pathognomonic of Leydig cells (LCs); they are thought to be rare in normal testes and to occur only in approximately one third of LC tumors. We noticed that crystals present in touch imprint and frozen sections of an LC tumor disappeared after tissue fixation. This phenomenon led us to hypothesize that their reported low frequency in normal and neoplastic LCs may be secondary to degradation/dissolution of the crystals after formalin fixation. Our review of the literature also led us to hypothesize that RC are better preserved after air-drying and alcohol fixation. We collected testicular samples from 21 autopsies including air-dried cytologic preparations and tissue samples that were fixed in alcohol or formalin. We found that RC are common in normal LC but dissolve rapidly in formalin and slowly and only partially in alcohol. The composition of RC is unknown; however, they have been reported to stain specifically for nestin, an intermediate filament expressed mainly in neural and muscle tissue. Because the crystals have only been described in androgen-producing cells, we hypothesized that the crystals may represent a crystallized form of androgenic hormones, hormone complexes, or enzymes involved in their synthesis. We performed immunostains for androgens and enzymes involved in androgenesis. We also performed nestin immunostain to confirm the previous study. The crystals stain specifically with antibodies anti-3ß-hydroxysteroid dehydrogenase and are negative for the remaining androgenic enzymes, androgenic hormones, and nestin.