bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pulmonary Granuloma Associated with Pneumocystis Jirovecii in a Patient with Peripheral T-Cell Lymphoma: Presentation of a Rare Case with Review of the Literature
Authors: Aneja A , Arriola A , Jhala A 
Published: October 2020

Abstract
Casestudy: Although Pneumocystis jirovecii (PJ) is a known opportunistic pulmonary infection among immunocompromised patients, it is a rare (&lt;5%) cause of pneumonia in this cohort. Majority of cases are observed in patients with HIV or solid organ transplant recipients. Granuloma formation in patients infected with PJ is even rarer. We present a case of multiple pulmonary granulomas associated with PJ in a non-HIV patient. A 49-year-old female with a history of asthma presented with worsening dyspnea. Initial imaging revealed multifocal bilateral areas of consolidation and a diagnostic bronchoalveolar lavage (BAL) demonstrated PJ organisms. Additional investigation was pursued to determine the etiology of this infection in a seemingly immunocompetent patient. New findings noted during hospitalization included a new maculopapular skin rash, significant weight loss, and lymphopenia. A bone marrow biopsy revealed a clonal proliferation of plasma cells. Subsequent imaging revealed mediastinal adenopathy. There was no improvement in symptoms despite treatment for pneumonia and a month later, a lung wedge biopsy revealed organizing acute lung injury and multiple non-caseating granulomas without lymphoid rimming, asteroid, or Schaumann bodies. Silver stain highlighted numerous PJ organisms within and outside of the granulomas. An inguinal lymph node and skin biopsies eventually revealed involvement by peripheral T-cell lymphoma (PTCL). Repeat BAL revealed clearance of organisms after additional therapy; however, the PTCL was refractory to chemotherapy and palliative care was pursued. To date, we could only find &lt;30 cases of pulmonary PJ granulomas in patients with hematologic malignancies. Most are in patients with B cell lymphomas and just 1 report in a patient with adult T-cell leukemia/lymphoma. In summary, this case highlights that PJ associated pulmonary granulomas are uncommon, can be a harbinger of an underlying immunocompromised state, such as a lymphoma, and are even rarer to be associated with PTCL.
Toxic Adenoma Mimicking Papillary Thyroid Carcinoma
Authors: Aksionau A , Wei E 
Published: October 2020

Abstract

Introduction/Objective
A thyroid nodule requires multiple steps in the identification of its nature, which determines its management. In most cases, low TSH levels support a benign origin. However, examples of hyperfunctioning thyroid carcinoma have been reported. Recent observations show that the number of thyroid cancer increases with the prevalence in young patients. The ambiguity of some cases necessitates the use of a full range of diagnostic methods up to molecular cytological diagnostics.

Methods
We present a report on the case of an 11-year-old boy complaining of weight loss despite having a good appetite.

Results
The blood test showed free T4 1.51 (0.81 - 1.35 ng/dL), free T3 6.04 (3.31 - 4.88 pg/mL), and TSH 0.01 (0.662 - 3.90 mU/L). During sonography, the left thyroid lobe was almost completely replaced by an isoechogenic circumscribed mass with multiple colloid cystic spaces. No suspicious microcalcifications or abnormal cervical lymph nodes were identified. A thyroid scan revealed the presence of a toxic autonomously functioning thyroid nodule in the left lobe. Methimazole and propranolol were prescribed, with subsequent left hemithyroidectomy on 10/21/2019.
Grossly, the left thyroid lobe was 3.4 x 2.5 x 2 cm. The sectioning revealed light brown homogeneous stroma with a well-defined yellow-tan nodule (1.1 x 1.1 x 1 cm) in the middle of the lobe. The specimen was entirely submitted for permanent fixation in formalin. Microscopically, an encapsulated nodule was seen with the follicular and focal papillary pattern; nuclear features strongly and multifocally suggested a diagnosis of papillary carcinoma. HBME1 and calcitonin immunostains were negative; CK19 immunostain was multifocally positive. Next-generation sequencing supported the diagnosis of toxic adenoma with atypical features.

Conclusion
Every case of thyroid neoplasm should be fully investigated using modern and high-tech technologies, regardless of the functioning state of the gland. Molecular testing is supportive, especially in ambiguous situations, which benefits the patient.
Endobronchial Mucus Gland Adenoma: A Case Report
Authors: Sandhu A D , Liu Y 
Published: October 2020

Abstract

Introduction/Objective
Mucus Gland Adenoma (MGA) is a rare benign epithelial tumor with few case reports. They arise from submucosal glands and ducts of large proximal airways, but have been reported peripherally and within lung parenchyma. MGAs form an exophytic mass that causes obstructive symptoms, occurring in all ages without gender predilection. MGAs have low to no malignant potential, but may be confused with more aggressive entities. Thus, MGA may present a diagnostic challenge on frozen section.

Methods
We present the case of a 66-year-old male with recurrent right pneumonia and empyema. Bronchoscopy revealed an obstructing mass in the right bronchus intermedius. Biopsies and cytology were insufficient showing only benign bronchial epithelium. Therefore, the patient underwent surgical sleeve resection of the right bronchus intermedius for diagnosis and treatment.
It was received in two pieces measuring 1.0 x 0.6 x 0.5 and 2.0 x 1.5 x 0.9 cm - a well-circumscribed, firm white nodule with a smooth exterior and pushing borders. Cut surfaces were mucoid, homogenous and rubbery, with cysts measuring up to 2 mm. Histology showed crowded mucus-filled acini and tubules lined by bland cuboidal to columnar cells without atypia or mitosis. The stroma showed smooth muscle bundles and lymphocytic infiltrate. Benign bronchial epithelium lined the surface. A preliminary diagnosis of “adenoma” was made, with final classification pending permanent section.

Results
It showed positivity for s100, CK5/6, and CAM5.2. DOG1 showed a luminal staining pattern. NapsinA was negative and TTF-1 was patchy. P63, P40, CK5/6 and calponin highlighted myoepithelial cells, underlining the benign nature of the process.

Conclusion
MGA may resemble adenocarcinoma, low-grade mucoepidermoid carcinoma, and other benign adenomas. It presents a diagnostic challenge on frozen section as its malignant differentials are more common. Thus, it is important to recognize and be aware of these rare, benign tumors.
Concurrently Presenting Endometrial And Ovarian Endometrioid Adenocarcinomas- A Clinicopathologic Study Of 52 Cases
Authors: Lahori M , Borazan N 
Published: October 2020

Abstract

Introduction/Objective
Concurrent presentation of endometrioid adenocarcinomas in endometrium and ovary is uncommon, but oft-noted. They may arise from ovarian metastasis of endometrial carcinoma, endometrial metastasis of ovarian carcinoma, or synchronous development of independent primary lesions. Scully and Young criteria have been widely used to differentiate the site of origin.1 Role of a field effect in the genesis of synchronous carcinomas is supported by increasing evidence that endometrioid ovarian carcinomas arise from endometriosis. 2–5

Methods
All cases of concurrent endometrioid adenocarcinomas of the endometrium and ovary between 2002 and 2019 in the Mount Sinai health system New York were included, using pathology database (Powerpath) and EMR database (Epic).

Results
52 cases of concurrent endometrioid adenocarcinomas of endometrium and ovary were identified between 2002 and 2019. Mean age at presentation was 54.07 years (24–80). 69.2% had synchronous origin, 21.15% had endometrial primary and 3.8% had an ovarian primary. Ovarian endometriosis was identified in 51.9% and complex atypical hyperplasia in 26.9%. Lower uterine segment involvement was seen in 26.9%, bilateral ovarian involvement in 40.3%, fallopian tube involvement in 23% and lymphovascular space invasion in 25%. Different histologic grade of ovarian and endometrial counterparts was noted in 25% cases. Synchronous endometrioid carcinomas had the following characteristics: mean age at presentation 50.6 years, associated with CAH in 33.3% and endometriosis in 66.6%, presentation at stage 1 in 63.8% and lower grade of differentiation (grade 1/2) in 80.5% cases.

Conclusion
In majority of cases, synchronous endometrioid carcinomas have a younger mean age at presentation, are lower grade tumors, present at stage 1 and have associated endometriosis. It can be inferred that the percentage of synchronous tumors with endometriotic foci would be even higher (with extensive sampling & assuming that the neoplastic process has replaced native benign endometriotic foci) – pointing towards the likely role of endometriosis in the genesis of these tumors.
Impact of the Cholangiocarcinoma Component of Combined Hepatocellular/Hepatocholangiocarcinoma on Patient Survival
Authors: Gubbiotti M , Furth E E , Fortuna D , Tondon R , Karasic T 
Published: October 2020

Abstract

Introduction/Objective
The radiopathologic correlation, overall prognosis, and best management of combined hepatocellular carcinoma/cholangiocarcinoma (HCC/CC) remains uncharted territory.

Methods
We studied whether the percentage (%) of the cholangiocarcinoma (CC) component of HCC/CC impacted patient survival. A retrospective analysis was performed including all cases (18) of HCC/CC in the pathology archives from 2011–2019. Tumor beds were submitted entirely in all but 2 specimens, which were representatively sampled.
Slides from these cases were reviewed to assess % of CC. Survival was calculated from the date of diagnosis to the date of demise and censored as the date last known alive at the time of the study.

Results
13 cases were liver explants, 2 were resections, and 3 were biopsies. 13 cases were from clinical stage II patients, 2 were clinical stage IVA, and 2 were clinical stage IVB. All but one patient in the study group were males. There was no predilection for any specific tumor site. The cases were divided into three groups related to CC percentage: 0–20 (n=5), 21–40 (n=6), and &gt;40% (n=7). AFP was variably elevated in select cases, with the highest values found in the medium and high % groups. Though there was no significant difference in overall survival among the three groups, mean and median survival trended in a downward fashion with increased % CC. Interestingly, radiology read 12 cases as HCC, 1 as intrahepatic CC, and only 1 as cholangiocarcinoma/hepatoma, atypical for HCC.

Conclusion
We conclude that there is a trend for decreased survival as % CC increases in combined HCC/CC patients, with patients with &gt;20% CC tending to have lower survival rates. Furthermore, incongruity exists between radiologic and pathologic diagnoses of combined HCC/CC. Additional study with a larger sample size will yield more information to understand better prognosis as well as guide more accurate diagnosis of these patients.
Checkpoint Inhibitor Colitis Masked by Multi-drug Effect
Authors: Davis J W , Canevari M , Shaw J C 
Published: October 2020

Abstract
Casestudy: The utilization of checkpoint inhibitors such as programmed cell death protein 1 (PD-1/PD-L1) inhibitors (nivolumab) and cytotoxic T-lymphocyte antigen 4 inhibitors (ipilimumab) for treatment of certain malignancies has steadily gained popularity. Medication related colitis is uncommon, with a reported incidence of 1–9% depending on the checkpoint inhibitor used, and the histologic features have been characterized in recent literature. Because of the immunomodulating effect of these drugs, infectious colitis is in the differential diagnosis of enteritis. Multi-drug therapy in many of these patients further complicates identification of the culprit drug. We present the case of a 63-year-old male with metastatic renal cell carcinoma being treated with both nivolumab and ipilimumab who presented with acute on chronic non-bloody diarrhea. His clinical course was complicated by hypotension, acidosis and coagulopathy. The clinical differential for his colitis was cytomegalovirus infection versus a checkpoint inhibitor colitis. Colonoscopy revealed continuous circumferential loss of vascularity and diffuse erythema throughout the colon. Histology showed acute colitis with prominent apoptosis, cryptitis, crypt abscesses, and rare ringed mitotic figures, but without architectural distortion. Occasional smooth purple crystals consistent with pill material were present in the mucosa, but without significant tissue reaction. No pathogenic organisms were identified, and a cytomegalovirus immunostain was negative. These histologic findings in concert with the clinical history are consistent with checkpoint inhibitor colitis and multi-drug effect. A review of the patient’s chart showed cholestyramine was added to the patient’s regimen during hospitalization, which was consistent with the morphologic appearance of the crystals. Given the acute complications of checkpoint inhibitor induced enterocolitis and potential for increased morbidity (rare cases of bowel perforation and subsequent resection), accurate diagnosis is imperative. Management of checkpoint inhibitor associated colitis ranges from initiation of immunosuppression to checkpoint inhibitor cessation. When these findings are masked by multi-drug effect, accurate diagnosis can be difficult.
Squamous Cell Carcinoma Of Pancreas: An Exceedingly Rare Entity In A Young Female
Authors: Arshi J , Rao D 
Published: October 2020

Abstract

Introduction/Objective
Squamous cell carcinoma(SqCC) of Pancreas is a vanishingly rare neoplasm with dismal survival. Only a handful of cases are ever reported as pure SqCC reportedly accounts for &lt;1% of malignancies of exocrine pancreas. Although, few theories have been proposed in an attempt to explain origin of this tumor including the possibility of (1)common progenitor cell and (2)squamous metaplasia, none have been proven with concrete evidence.

Methods
Here, we are presenting a case of squamous cell carcinoma of pancreas in a 33-year-old female who presented with 2-year history of right upper quadrant abdominal pain and a palpable mass. The imaging showed a multi-lobulated cystic mass encasing cystic duct, portal vein, and superior mesenteric artery.

Results
Grossly the mass was 5.2 cm in the greatest dimension. The mass revealed solid and cystic areas and was filled with hemorrhagic debris. On microscopy, the cyst wall was infiltrated by malignant neoplasm composed of irregular tongues and cords of malignant cells with nests of central keratinization. The tumor was consistent with squamous cell carcinoma. Foci of high grade cells with oval to spindled hyperchromatic nuclei, with many prominent nucleoli were also noted. There was no evidence of glandular component despite adequate sampling. The differential diagnoses of pancreatoblastoma and metastatic squamous cell carcinoma were considered and excluded.

Conclusion
SqCC of Pancreas is an exceedingly rare neoplasm of Pancreas. The diagnosis is made after extensive work up, adequate sampling and ruling out other common primary tumors and the possibility of metastasis. It usually follows an aggressive course with resistance to conventional neoadjuvant therapy. During the recent years, survival rates have shown some improvement with respectability and low to intermediate grade emerging as favorable prognostic factors. However, to further validate such findings noted in published case reports of this rare entity, collaborative studies with pooled data are deemed necessary.
A Rare Case Of Solid Pseudopapillary Neoplasm (Spn) Of Pancreas In A Male Patient
Authors: Arshi J , Rao D 
Published: October 2020

Abstract

Introduction/Objective
SPN is a rare neoplasm of the pancreas with low malignant potential and unknown histologic origin. It has a strong preponderance in young females (Female:Male ratio = 9:1).

Methods
We present a case of a 32-year-old morbidly obese male, who presented at our institution with an incidental large heterogeneous mass in the distal pancreas on imaging. Distal pancreatectomy with splenectomy was performed.

Results
Grossly, the mass measured 8 centimeters in greatest dimension with a tan-brown variegated cut surface.
The microscopic sections revealed a well-encapsulated tumor comprised of solid and cystic components. The solid areas show sheets and nests of hyalinized vascular stroma surrounded by small uniform cells with eosinophilic cytoplasm and bland round-to-ovoid nuclei. The immunohistochemical (IHC) stains showed variable positive expression with CD10, beta-catenin, and progesterone receptors. We also stained for newer putative IHC markers including LEF-1, TFE-3, SOX-11, and AMACR. Among them, LEF-1 was the only marker that showed 100% strong and diffuse, nuclear positive expression while the other three showed variable positive expression. These novel results are noted for the first time and is in congruence with the emerging molecular diagnostic finding that LEF-1 expresses the β-catenin binding domain of Wnt/β-catenin signaling pathway involved in the histologic origin of this tumor.

Conclusion
We present a rare case of SPN in a male patient. Additionally, this is the first case to report the staining pattern of newer putative markers of LEF-1, TFE-3, SOX-11, and AMACR that may suggest the pathobiology of histologic origin of this tumor.
Mistaken Identity: A Rare Tumor Unexpectedly Identified in a Renal Transplant Recipient’s Obsolescent Native Kidney
Authors: Ibrahim D , Dominiak N 
Published: October 2020

Abstract
Casestudy: Multilocular cystic clear cell renal cell neoplasm of low malignant potential is a rare entity, comprising 1–2% of all renal tumors. This is mostly due to that the majority (90%) are incidentally found by radiologic exams performed for other reasons. Cystic lesions are not uncommon in patients over 50 years old and are difficult to assess surgically. Therefore, it is even more important to recognize this subtype of renal cell neoplasm histopathologically, as these tumors behave very differently from their other counterparts. We report a case of a 63- year-old female renal transplant recipient who subsequently presented with bilateral native nephrolithiasis and left flank pain. Imaging demonstrated the calculi, for which the patient underwent left nephrectomy to relieve her pain. On gross examination, the cut surface of the kidney revealed a well-circumscribed, well-encapsulated, multiloculated cystic lesion at the upper pole measuring 1.9 cm with thin fibrous septae. Microscopically, the lesion was composed of loculations and septations as were observed grossly. These spaces were lined by one to two layers of cells with clear cytoplasm, some of which were present within the septal walls. The nuclei were haphazardly distributed within the cytoplasm of the cells. They were grade 2 according to the International Society of Urologic Pathology grading scale. The tumor cells demonstrated CK7 positivity. To our knowledge, this is the first case of multilocular cystic clear cell renal cell neoplasm of low malignant potential in a transplant recipient’s native end stage kidney mistaken for renal calculi.
A Rare Case of Retroperitoneal Isolated Enteric Duplication Cyst with Malignant Transformation
Authors: Alhamar M , Alkamachi B , Jaratli H , Shah R 
Published: October 2020

Abstract

Introduction/Objective
Isolated enteric duplication cysts (IEDC) are rare congenital abnormalities that do not have a communication with the normal intestinal lumen. They are usually recognized before the age of 2 years. They are rarely encountered in adults and very seldom undergo malignant change. These cysts are predominantly located within the abdominal cavity. They histologically demonstrate gastrointestinal mucosal lining and smooth muscle in the wall.

Methods
We report a case of a 45-year-old African American female who was evaluated for a pain in the left-sided abdomen and pelvis. Magnetic resonance imaging revealed a large multilocular cystic retroperitoneal mass, extending into the left iliac fossa and anteriorly displacing the colon. No other mass lesion was radiologically identified. The main radiologic impression was sarcoma. A biopsy was performed, and a pathologic diagnosis of adenocarcinoma was established with a consideration of primary retroperitoneal mucinous cystadenocarcinoma. An en bloc surgical resection of the retroperitoneal mass with adjacent segment of the descending colon was accomplished. Gross examination revealed an 11 x 5 x 4 cm cystic mass, containing a necrotic fluid and adherent to a 4 cm long segment of descending colon. Microscopically, the mass was lined by neoplastic cells arranged into tubules, villi and glands. Areas of necrosis were present. The wall of the cystic mass showed extensive hyalinized fibrosis and focal invasion. The neoplastic cells had positive immunoreactivity for CK7, CK20 and CDX2, but were negative for PAX8, PR and ER. Desmin immunostain and trichrome special stain highlighted a focus of smooth muscle bundle within the wall of the mass. These findings led to the pathologic diagnosis of adenocarcinoma arising in retroperitoneal IEDC.

Conclusion
In conclusion, we present this case because of its unusual presentation and location. Recognition of this entity and its rare malignant potential is important as it can pose a diagnostic challenge to pathologists.
A Case of Small-Cell Neuroendocrine Carcinoma of the Gallbladder in a Background of High-Grade Biliary Intraepithelial Neoplasia
Authors: Alkamachi B , Sharaf Aldeen B , Alhamar M , Jaratli H 
Published: October 2020

Abstract

Introduction/Objective
Neuroendocrine carcinoma (NEC) of the gallbladder is a very rare neoplasia comprising 4% of all malignant gallbladder neoplasms. Most of NECs show molecular accumulation of TP53, while KRAS mutations are rare. Approximately 40% of Biliary intraepithelial neoplasia (BilIN) cases are associated by KRAS mutations as an early molecular event, whereas TP53 mutation appears to be a late molecular event.

Methods
We report a case of a 62-year-old male who presented to emergency department for evaluation of abdominal pain associated with vomiting. Imaging studies demonstrated a distended gallbladder with small amount of pericholecystic fluid along with 5 mm calculus at the gallbladder neck consistent with symptomatic cholelithiasis and acute cholecystitis. No mass lesion was radiologically identified. A laparoscopic cholecystectomy was performed.
Grossly, there was a thickened area of mucosa at the distal body-fundus, measuring 5.0 x 4.5 cm. Multiple calculi were present. On histology, thickened area showed sheets and nests of moulded small cells with hyperchromatic nuclei, brisk mitotic activity and confluent necrosis. A diagnosis of small-cell NEC is established, and confirmed by positive immunoreactivity to neuroendocrine markers (CD56, Synaptophysin) and epithelial markers (CK7 and CAM5.2). Multiple foci of high-grade BilIN were noted. Subsequent entire submission of the gallbladder revealed no adenocarcinoma present. The pathologic stage was pT2a pNX.

Conclusion
In conclusion, this rare gallbladder carcinoma is found incidentally in a patient with acute cholecystitis symptoms and no clinical proof of mass lesion. In addition, there is a background of high-grade BilIN, which shares the molecular pathway of small-cell NEC. Therefore, awareness of such coexistence of these two pathologic entities in the same gallbladder, is essential to alert pathologists to look for the poorly-prognosis small cell NEC, whenever BilIN is encountered in random section of a gallbladder.