Claudia P. Rojas

Searching for mammary analog secretory carcinoma of salivary gland among its mimics

Mammary analog secretory carcinoma of salivary gland is a recently described entity with unique morphologic, clinical, and genetic characteristics, including the characteristic t(12;15)(p13;q25) with ETV6-NTRK3 translocation found in secretory carcinomas of the breast. Before their initial description, these salivary gland tumors were generally diagnosed as acinic cell carcinoma or adenocarcinoma. For the purpose of this study, all cases of salivary gland acinic cell carcinoma, cribriform cystadenocarcinoma, and adenocarcinoma, not otherwise specified (NOS), diagnosed over a 10-year period were retrieved from our surgical pathology files. There were a total of 11 cases diagnosed as acinic cell carcinoma, 10 cases of adenocarcinoma, NOS, and 6 cases of cribriform cystadenocarcinoma. All slides were reviewed by two pathologists (AP, CGF) and tumors that show morphologic features of mammary analog secretory carcinoma according to the recent literature were selected. This process narrowed down the initial number to six cases originally diagnosed as acinic cell carcinoma, three cases originally diagnosed as adenocarcinoma, NOS, and one case originally diagnosed as cribriform cystadenocarcinoma. The 10 cases were subjected to immunohistochemistry for S-100, mammaglobin, and ANO1, as well as fluorescence in situ hybridization analysis for t(12;15)(p13;q25) with ETV6-NTRK3 fusion rearrangement. The ETV6-NTRK3 gene rearrangement was detected in three tumors. These three tumors, initially diagnosed as acinic cell carcinomas, stained positive for S-100 and mammaglobin, and negative for ANO1 by immunohistochemistry. Two of the three patients were male (2/3). In summary, mammary analog secretory carcinoma is a newly described diagnostic entity that should be in the differential diagnosis of salivary gland tumors that morphologically mimic other neoplasms, mainly acinic cell carcinomas. They differ from conventional acinic cell tumors immunohistochemically and molecularly. Positivity for mammaglobin and S-100, and negativity for ANO1 are useful screening tools before confirmatory molecular studies.

Low biopsy volume in ureteroscopy does not affect tumor biopsy grading in upper tract urothelial carcinoma

OBJECTIVES Urothelial carcinomas (UC) from the upper urinary tract represent 7%-10% of all kidney malignancies. With current ureteroscopic (URS) techniques, small tissue samples are usually the only available histopathologic material for evaluation, representing a diagnostic challenge. Precision in diagnosis is essential for treatment decision making. There has been much debate as to whether tumor grade and stage found on biopsy agree with final pathology. The purpose of this study is to evaluate whether URS biopsy volume affects tumor grading and staging agreement between biopsy and nephroureterectomy (NU) specimens. MATERIALS AND METHODS We reviewed 137 URS biopsies in 81 patients with suspected upper urinary tract UC performed from April 2002 to April 2011. Of those, 54 patients had both the URS biopsy and NU performed at our institution and were available for review. Biopsy dimensions were recorded to calculate estimated ellipsoid volume, and 2 urological pathologists independently evaluated histologic grade (ISUP/WHO 2004), (based on pleomorphism and mitosis) and depth of invasion. Statistical analysis was performed to evaluate URS biopsy and NU specimen grade and stage concordance. In addition, univariable and multivariable analyses was performed to assess the effect of biopsy volume on agreement. RESULTS Of the 54 patients studied, low grade and high grade UC biopsy were found in 8 (15%) and 46 (85%), URS biopsies, respectively. Regarding biopsy stage, 51 (94%), 1 (2%), and 2 (4%) were stage Ta, T1, T2, respectively. Grade concordance was 92.6%, (95% CI: 82.4%-98.0%). Stage concordance was 43% (95% CI: 28.7%-55.9%). Multivariable analysis showed biopsy volume did not affect tumor assessment of grade (P = 0.81) or stage (P = 0.44). CONCLUSIONS Histologic grade assigned on the URS biopsy sample accurately predicts histologic grade in the resected specimen (92.6%), even when the biopsy volume is small. Grading in URS biopsies provides sufficient information for clinical decision making that is independent of sample volume.

A child with BK virus infection: Inadequacy of current therapeutic strategies

Pereira T, Rojas CP, Garcia‐Buitrago MT, Chandar J, Abitbol C, Seeherunvong W, Rusconi P, Bruce JH, Zilleruelò G. A child with BK virus infection: Inadequacy of current therapeutic strategies. 
Pediatr Transplantation 2011.

Rare presentation of BK encephalitis in a child: imaging and pathological findings

Clinically apparent BK virus infections mostly involve the genitourinary system of immunocompromised patients, particularly those who have undergone renal or bone marrow transplantations. Central nervous system involvement is extremely rare. In this report, we describe the pathologic and MR findings of BK encephalitis in a child. Initial involvement of the brainstem and the cervicomedullary junction was noted that later rapidly progressed to involve other regions of the supratentorial brain parenchyma including the basal ganglia and the thalami as well as the cerebellum.

Pulse granuloma of the rectum: an unusual entity.

Sir: Pulse granuloma is an unusual, foreign body type of inflammatory reaction to the cellulose moiety of plant matter instead of the starch granules. It is more commonly noted in the oral cavity, with few cases having been reported in the lung and other unusual sites such as the nasal cavity, fallopian tube, and ovary. Here, we report a case of a human immunodeficiency virus (HIV)-positive patient with a pulse granuloma located in the rectum. The histopathological features of a vegetable granuloma are very characteristic, and are therefore helpful in making a confirmatory diagnosis, without the use of any special studies. Hence, by means of this case report, we emphasize that the complete understanding and awareness of this pathological entity is mandatory. This can help in the early recognition and treatment of these patients. Microscopic examination remains the gold standard for its diagnosis, which helps the pathologist to reach a definitive diagnosis with minimal delay, and also to avoid the use of unnecessary, time-consuming investigations. A 53-year-old HIV-positive male presented with the complaint of intermittent passage of bright red blood from the rectum, along with a burning sensation. There were no associated complaints. He was a known hypertensive, and also had hyperlipidaemia. He gave a past history of an appendectomy and incisional hernia repair many years previously. On colonoscopy performed in 2011, left-sided diverticulosis with internal haemorrhoids was identified. On examination, vital signs were within normal limits. A small verrucous lesion was seen on the posterior midline of the anus, and digital rectal examination revealed another small flat, hard lesion on the anterior distal part of the rectal wall. On anoscopy, this lesion appeared to be mostly submucosal, and showed some irregularity of the mucosa. The patient underwent excision and fulguration of these abnormal lesions. On histopathological examination of the haematoxylin and eosin-stained slides of the rectal specimen, the colonic mucosa showed granulation tissue, acute and chronic inflammation, foreign body giant cells, submucosal and intramural identifiable particles of vegetable matter, and hyaline rings consistent with rectal pulse granuloma (Figures 1 and 2). Pulse granulomas have been given different designations by various authors, including names such as giant cell hyaline angiopathy, pulse granuloma, oral vegetable granuloma, hyaline ring granuloma, foodinduced granuloma, and granuloma with giant cells and hyaline change. On review of the literature, we could find only two cases of rectal pulse granuloma, one arising from the rectum itself, and the other in a rectocutaneous fistula. PubMed was used for the literature search.

Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposis sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias.

Intraductal papillary neoplasm of the bile ducts: A case report and literature review.

Intraductal papillary neoplasm of the bile duct (IPNB) is a rare bile duct neoplasm mostly found in far eastern nations where hepatolithiasis and clonorchiasis infections are endemic. In western countries, it is very rare and the etiology is unknown. In this article, we report the first IPNB patient we encountered in our clinic and a literature review. The patient is a 38-year-old female with a history of choledocholithiasis who presented with obstructive jaundice. She was found to have a papillary mass at the junction of the right hepatic duct and common hepatic duct with six masses in the liver parenchyma. The immunophenotypic and histologic features of the tumor are consistent with IPNB, gastric subtype. The patient had a partial hepatectomy and has been receiving palliative chemotherapy. In a search of PubMed database, we collected 354 IPNB patients reported in 22 articles. In these patients, 52.8% were from Japan and 27.7% were from western countries including the United States (11.0%). The age of the patients ranged from 35 to 80 years old with an average of 64.6. Male/female ratio was 1.5. Macroscopically, 57.5% of the tumors were in the left lobe and 29.5% were in the right lobe. The average size of the tumor were 4.2 cm at the time of diagnosis. Histologically, pancreato-biliary subtype accounted for 41.8%, intestinal 28.0%, gastric 13.5% and oncocytic 16%. An invasive component is most often present in the pancreato-biliary and gastric subtypes. Despite recent advanced technologies, diagnosis of IPNB is still challenging, especially in western countries due to its rarity. Defined clinico-pathologic features are in demand for the accurate diagnosis and proper treatment.

Angiosarcoma of the Pelvis in a 13-Year-Old Girl: Case Report and Review of the Literature

Angiosarcomas are highly aggressive malignancies of vascular origin and are very rarely found in children. We report a case of a 13-year-old girl with a history of abdominal pain and increased abdominal girth. Radiologic imaging showed significant ascites and large pelvic masses involving bilateral adnexa with abdominal spread. Microscopic examination of a biopsy revealed pleomorphic epithelioid and spindle cells with brisk mitotic activity, intracytoplasmic vacuoles, vascular channels, and large areas of hemorrhage and necrosis. Immunohistochemistry analysis showed strong and diffuse positivity for CD31, D2-40, ERG, FLI-1, and focally for CD34, vWF, and EMA. The diagnosis of metastatic angiosarcoma was rendered. The patient was treated aggressively with systemic chemotherapy, immunotherapy, cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy, with a favorable response after 1-year follow-up. Angiosarcoma should be considered when encountering a vascular tumor with pleomorphism, brisk mitotic activity, and necrosis. Immunohistochemistry studies are necessary for proper diagnosis.

Strongyloides Colitis as a Harmful Mimicker of Inflammatory Bowel Disease

Autoinfection caused by Strongyloides stercoralis frequently becomes a life-long disease unless it is effectively treated. There is overlapping histomorphology between Strongyloides colitis and inflammatory bowel disease; a low index of suspicion can lead to misdiagnosis and fatal consequences. We present a case of Strongyloides colitis mimicking the clinical and pathologic features of inflammatory bowel disease. A 64-year-old female presented to the emergency department with a four-day history of abdominal pain, diarrhea, and hematochezia. Colonoscopy revealed diffuse inflammation suggestive of inflammatory bowel disease, which led to initiation of 5-aminosalicylic acid and intravenous methylprednisolone. Biopsies of the colon revealed increased lymphoplasmacytic infiltrate of the lamina propria with eosinophilic microabscesses and presence of larvae, consistent with Strongyloides stercoralis. Immunosuppressive medication was halted. The patient ultimately died a few days later. This case emphasizes the importance of identifying the overlapping clinical and pathologic features of Strongyloides colitis and inflammatory bowel disease. A high index of suspicion and recognition of particular histological findings, including eosinophilic microabscesses, aid in the correct diagnosis. Definitive diagnosis is crucial as each disease carries distinct therapeutic implications and outcome.

Metastatic prostatic adenocarcinoma diagnosed in a bronchoalveolar lavage specimen: An unusual presentation of a common tumor.

Metastatic prostatic adenocarcinoma presenting as a primary lung disease is rare. We present a 52-year-old male with a 3-month history of cough, shortness of breath, and weight loss with clinical and radiological findings suggestive of a primary lung disease: Bilateral interstitial and alveolar opacities with blunting of the costophrenic angles, multiple diffuse foci of consolidations and nodules, predominantly subpleural and located in the lower lobes, and diffuse interlobular septal thickening and peribronchial thickening. The patient underwent bronchoscopy and bronchoalveolar lavage (BAL) was obtained. Cytospin smears were diagnostic for a low-grade adenocarcinoma. Clinically, the patient had elevated serum prostate-specific antigen (PSA) levels greater than 5,000 ng/mL. Because of this, immunocytochemistry for PSA was performed which was positive, confirming the diagnosis of metastatic prostatic adenocarcinoma. This unusual case of metastatic adenocarcinoma of the prostate first diagnosed by BAL highlights the significance of available clinical information and the use of immunocytochemistry for proper diagnosis.