Non-invasive Follicular Thyroid Neoplasm with Papillary-Like Nuclear Features (NIFTP) Lowers the Risk of Malignancy in the Bethesda System for Reporting Thyroid Cytopathology Diagnostic Categories.

Background: The introduction of non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) has been shown to decrease the risk of malignancy (ROM) in The Bethesda System for Reporting Thyroid Cytopathology. This knowledge may alter the management of patients with thyroid nodules. Objectives: To correlate cytological diagnosis with histological diagnosis for establishing the ROM of all Bethesda system categories after the introduction of NIFTP. Methods: This was a retrospective cohort study. All consecutive fine-needle aspiration cytology (FNAC) specimens collected from January 1, 2013, to December 31, 2017, at King Abdullah Medical City, Jeddah, Saudi Arabia, were assessed, and patients who underwent surgical excision of thyroid nodules were further analyzed. The ROM and overall ROM for each Bethesda category were calculated with and without considering NIFTP as a malignant tumor. Results: Overall, 1066 FNAC specimens were collected, of which 281 had a surgical correlation. Our cases included 18 (6.4%) non-diagnostic (ND), 109 (38.8%) benign, 28 (9.9%) atypia/follicular lesion of undetermined significance (AUS/FLUS), 39 (13.8%) follicular neoplasm or suspicion for follicular neoplasm (FN/SFN), 20 (7.1%) suspicion for malignancy (SM), and 67 (23.8%) malignant (POM) cases. After considering NIFTP diagnosis on resection specimens, the ROM decreased as follows: ND, 38.8% to 27.7% (P = 0.2388); benign, 21.1% to 11.9% (P = 0.0343); AUS/FLUS, 50% to 39.2% (P = 0.2089); FN/SFN, 53.8% to 33.3% (P = 0.0336); SM, 85% to 75% (P = 0.2147); POM, 95.5% to 88% (P = 0.0582). Conclusion: The introduction of NIFTP would significantly decrease the ROM of thyroid FNAC in both benign and FN/SFN categories of the Bethesda system.

Atypical hemolytic-uremic syndrome due to complement factor I mutation.

Atypical hemolytic-uremic syndrome (aHUS) is a rare disease of complement dysregulation leading to thrombotic microangiopathy (TMA). Renal involvement and progression to end-stage renal disease are common in untreated patients. We report a 52-year-old female patient who presented with severe acute kidney injury, microangiopathic hemolytic anemia, and thrombocytopenia. She was managed with steroid, plasma exchange, and dialysis. Kidney biopsy shows TMA and renal cortical necrosis. Genetic analysis reveals heterozygous complement factor I (CFI) mutation. Eculizumab was initiated after 3 mo of presentation, continued for 9 mo, and stopped because of sustained hematologic remission, steady renal function, and cost issues. Despite this, the patient continued to be in hematologic remission and showed signs of renal recovery, and peritoneal dialysis was stopped 32 mo after initiation. We report a case of aHUS due to CFI mutation, which, to the best of our knowledge, has not been reported before in Saudi Arabia. Our case illustrates the challenges related to the diagnosis and management of this condition, in which a high index of suspicion and prompt treatment are usually necessary.

Pediatric Gastrointestinal Basidiobolomycosis: Case Report and Review of Literature.

Basidiobolus ranarum is an environmental saprophyte found worldwide. It is a member of the order Entomophthorales of the class zygomycete. The infection is generally restricted to subcutaneous tissues. However, the disease can involve visceral organs, causing gastrointestinal basidiobolomycosis (GIB). GIB usually has nonspecific clinical manifestations and its diagnosis is challenging. Missed and delayed diagnosis of GIB increases the risk of morbidity and mortality, especially in pediatric patient. Previously, most of the cases in Saudi Arabia have been reported from southern region (Tohama, Aseer Region); the current study reports cases from different regions. We report a case of severe GIB and four other cases of pediatric GIB in western region of Saudi Arabia. The aim of our report is to describe the wide range of clinical presentations, diagnosis, management and outcomes. Our case series includes the youngest case report of GIB, a 16-month-old infant and highlights two important points. First, the need of high index of suspicious for diagnosis of GIB and its inclusion in the differential diagnosis of any abdominal mass, fever and eosinophilia. Second, it gives additional supportive evidence that medical management with voriconazole without surgical intervention (intervention if the condition is diagnosed early) is curative.

Posterior fossa teratoma.

Germ cell tumors comprise approximately 2-5% of all childhood brain tumors. They arise predominantly in the pineal and suprasellar region, but may occur throughout the brain. Teratomas are generally divided into gonadal and extragonadal types. A posterior fossa teratoma is a rare occurrence. The focus of this discussion is a 5-year-old boy with posterior fossa teratoma who recovered completely after medical and surgical intervention. We also present his interesting imaging and pathological findings.

Primary prostatic central zone adenocarcinoma.

The central zone (CZ) of the prostate is embryologically, anatomically, and histologically distinct. High-grade prostatic intraepithelial neoplasia (HGPIN) and prostatic adenocarcinoma (PAC) are encountered in the CZ, but have not been well studied. Non-CZ PAC that spread into the CZ can mimic CZ PAC. We reviewed 300 consecutive radical prostatectomies performed for PAC to identify cases showing PAC and HGPIN in the CZ. There were nine PAC (3%) localized predominantly in the CZ, presenting as a single tumor nodule (8/9) and associated with 4.5+/-1.1 foci HGPIN in the CZ and with only 1.7+/-0.5 foci in the PZ. Of the 291 non-CZ PAC, 24 cases showed satellite tumor nodules in the CZ, and 92 cases demonstrated secondary contiguous spread to the CZ. As compared to the non-CZ PAC, CZ PAC tended to have lower tumor volume, but had higher Gleason scores (8.10+/-0.6 vs. 6.30+/-0.7, p<0.05), as well as a higher incidence of a ductal carcinoma component (6/9), higher rates of capsular penetration, positive resection margins (4/9), and seminal vesicle spread (2/9). The CZ HGPIN associated with CZ PAC demonstrated cells with prominent nucleoli and formed either slender papillary structures or cribriform/solid patterns. The correlating positive biopsy cores were from the mid portion or from base of prostate and contained foci of HGPIN in 4/7 cases. The CZ PAC is characteristically accompanied by more foci of HGPIN in the CZ than in non-CZ and is associated with high grade and high stage. Preoperative diagnosis of CZ PAC can be suspected due to the histopathological features in the biopsy and is important to improve the free surgical resection rate.

Secondary prostatic adenocarcinoma: a cytopathological study of 50 cases.

Positive diagnosis of metastatic prostate adenocarcinoma (PAC) can be made by microscopic examination of the cytologic specimens and immunostaining for prostate-specific antigen (PSA) and prostate acid phosphatase (PAP). Immunohistochemical markers have been known to display negative, weak, or focal staining in poorly differentiated PAC and in patients with prior hormonal and/or radiation therapy. The purpose of this study is to characterize the cytopathology of metastatic PAC as it has not been documented in large series. Fifty cases of metastatic PAC with cytological specimens consisting of 41 fine-needle aspiration biopsies (FNAB), 6 pleural fluid aspirates, and 3 catheterized urine samples were reviewed and correlated with the surgical specimens and the clinical charts. Immunostaining for PSA, PAP, cytokeratin AE1/3, cytokeratin 7 (CK7), cytokeratin 20 (CK20), vimentin, and carcinoembryonic antigen (CEA) was done. Mean patient age was 77 +/- 8 yr; serum PSA, 4.1 +/- 2.3; and primary PAC Gleason score, 8.1 +/- 1.5. Cytologically, the specimens consisted of cell clusters or cell sheets with overlapping uniform hyperchromatic nuclei with or without nucleoli. Twelve cases were not reactive to PSA and PAP and 44 cases displayed negative immunoreactivity to both CK7 and CK20. Carcinoid-like lesions and small cell carcinomas were seen in 4 cases and were misdiagnosed as nonprostatic origin based on the following features: negative immunoreactivity to PSA and PAP with or without positive reactivity to CEA, and different histopathological features when compared with the primary PAC. In addition to the frequency of high-grade PAC, awareness of the negative immunoreactivity to PSA and PAP, the discrepancy in the histopathological patterns between the primary and secondary tumors, especially the frequent neuroendocrine differentiation, are helpful features for the diagnosis of metastases of prostatic origin.