Scott Gilles

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-3β-hydroxysteroid dehydrogenase and are negative for the remaining androgenic enzymes, androgenic hormones, and nestin.

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.

Lines of Zahn in the Splenic Vein

Two years after a reduced-intensity umbilical cord blood haematopoietic cell transplantation for Philadelphia chromosome-positive B cell acute lymphoblastic leukaemia, a 62-year-old man presented with acute-onset right upper quadrant pain. Four weeks before this presentation, he had an episode of acute pancreatitis associated with disseminated zoster infection. This was demonstrated on an otherwise unremarkable, contrast-enhanced, abdominal computed tomography (CT) scan. A repeat abdominal CT scan at the time of the most recent presentation revealed an enlarged, diffusely hypodense spleen (suggestive of complete infarction) with lobulated contour and a non-occlusive distal splenic vein thrombus (►Fig. 1A, arrow), new from the prior study. There was no radiographic evidence of pancreatitis on this imaging. Positron emission tomography scan was confirmatory, showing no uptake by the spleen (►Fig. 1B). The patient underwent open splenectomy during which multiple adhesions in the peripancreatic/perisplenic area were found. Pathological examination showed an entirely infarcted spleen and thrombosed splenic vein showing lines of Zahn (►Fig. 2A, B). Venous location of the thrombus was confirmed also by an elastin stain (Verhoeff–Van Gieson), staining the adjacent artery but not the vein (►Fig. 2C, arrow). Extensive laboratory work up for malignancy, infection, autoimmunity and graft-versus-host disease was negative. A diagnosis of splenic infarction due to splenic vein thrombosis related to recent pancreatitis was established.

The role of bone marrow morphology in the diagnosis of relapsed acute myeloid leukemia

We read with interest the recent article by Zhou et al.1 in Leukemia. The authors first demonstrate an almost invariably abnormal multiparameter flow cytometry (MFC) when morphologic exam shows relapse in patients with acute myeloid leukemia (AML). This low false-negative rate of MFC questions the need for morphologic exam when MFC is negative and if the only question is whether the patient has relapsed.

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.

The unused iron

![Figure][1] A 35-year-old man with prolonged aplasia (62 days) after 2 inductions for acute myeloid leukemia (AML), underwent a myeloablative, matched-sibling, A-to-O allogeneic stem cell transplantation. Despite rapid neutrophil and platelet recovery, he remained red blood cell aplastic (

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 patients 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.