Kartik Viswanathan

The role of the Milan System for Reporting Salivary Gland Cytopathology: A 5-year institutional experience: Milan Classification System for Salivary Gland

Fine‐needle aspiration (FNA) is widely accepted in the preoperative management of salivary gland lesions. The proposed Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) aims to standardize reporting terminology. Studies regarding the risk of malignancy (ROM) for the proposed categories continue to evolve. The current retrospective study applied the MSRSGC to assess ROM for salivary gland lesions and focused on the “indeterminate” categories.

Juvenile Papillomatosis (Swiss-Cheese Disease) of Breast in an Adult Male With Sequential Diagnoses of Ipsilateral Intraductal, Invasive, and Widely Metastatic Carcinoma: A Case Report and Review of the Disease in Males

Juvenile papillomatosis of the breast (JPB, also known as Swiss cheese disease) is a rare ailment that typically afflicts young females, and presents as a mass-forming lesion. The lesional mass usually comprises multiple cysts and duct stasis, amid a variety of proliferative and nonproliferative epithelial changes. The proliferative changes include papillary hyperplasia, florid hyperplasia, and papillary apocrine hyperplasia. Concurrent carcinoma (either in situ or invasive) is present in approximately 10% of cases at presentation, and subsequent carcinoma (either in situ or invasive) is diagnosed in about 10% of patients. About 20% of patients have a strong family history of breast carcinoma. A total of 10 cases of JPB have been previously reported in males, both children and adults, only one of which, in a 33-year-old, was associated with invasive carcinoma. Here, another case of JPB in a 45-year-old male—one with subsequent sequential diagnoses of ipsilateral intraductal carcinoma, invasive carcinoma, and widely metastatic carcinoma over the course of 15 years—is reported.

Bilateral Multiple Mammary Myofibroblastomas in an Adult Male

In 2007, a 74-year-old male resident of New Mexico presented with bilateral breast masses. Notable history included cutaneous melanoma (status-post wide resection, in 1987) and prostatic adenocarcinoma (status-post radical prostatectomy, in 1992); without evidence of recurrence or metastases from either malignancy. Mammography showed 3 well-circumscribed, round, and homogeneous masses in the right (R) breast (largest 1.4 cm), and 2 similar masses in the left (L, largest 1.7 cm) (Figure 1A and B). These tumors were present in different quadrants, at variable distances from the respective nipples. Ultrasound examination revealed all 5 masses to be solid (Figure 1C and D). Needle core biopsies of the bilateral masses showed histologically similar spindle cell neoplasms. The lesional spindle cells displayed pale cytoplasm and elongated nuclei with micronucleoli, and were arranged in fascicles amid wispy straps of collagen. Neither nuclear atypia nor mitotic activity was evident (Figure 2). The spindle cells were immunoreactive for CD34, desmin, estrogen receptor, and Bcl-2 (Figure 3). Based on these findings, the diagnosis of bilateral multiple mammary myofibroblastomas (MM) was rendered. Clinical observation, and not surgical excision, was opted for. The subsequent 8 years were uneventful. In 2015, the patient was diagnosed to have mantle cell lymphoma. Computed tomography, performed as part of 735895 IJSXXX10.1177/1066896917735895International Journal of Surgical PathologyViswanathan et al research-article2017

Solitary Neurofibroma of the Breast, and “The Man From Istanbul” Syndrome

A 40-year-old asymptomatic woman, with neither medical nor family history of any pertinent disease, underwent her first breast screening evaluation. The mammogram showed unilateral focal ill-defined “asymmetry.” An ultrasound examination revealed the “asymmetry” to represent an ovoid 0.8 cm nodule in left breast at 12:00, 2 cm from nipple (Figure 1A). A needle core biopsy showed the lesion to comprise mainly of sheaves of twisted spindle cells set amid bands of rippling collagen and loose fibromyxoid matrix (lending a “shredded carrot” look; Figure 1B and C). Diffuse nuclear and cytoplasmic immunoreactivity for S100 protein (in the lesional Schwann cells) was evident (Figure 1D). Based on the aforesaid features, the diagnosis of neurofibroma (NF) was rendered. The patient desired removal of the lesion. The subsequently performed excision showed a pearly mass with a glistening cut surface. Histopathologically, the circumscribed but unencapsulated lesion was similar to that observed in the needle core biopsy (reactive changes of which were evident), and lay amid otherwise unremarkable breast tissue (Figure 1E and F). One year after excision, the patient showed no evidence of breast disease—either clinically or on imaging. Intramammary peripheral nerve sheath tumors— including NF and schwannomas—are uncommon. Intramammary NF typically occur as an asymptomatic and solitary lesion, although multiple mainly cutaneous-based ones can ensue in neurofibromatosis type 1. The macroscopic and microscopic appearances of NF are largely similar in all locations, although its myxoid variant has been described in the breast as elsewhere. The occurrence of NF in the breast exemplifies the phenomenon of a common tumor occurring in an uncommon location, and the diagnosis is straightforward “but only if the pathologist thinks of the possibility (a good example of Dr Ackerman’s “The man from Istanbul” syndrome). The latter 728337 IJSXXX10.1177/1066896917728337International Journal of Surgical PathologyCheng et al research-article2017

Fine needle aspiration of pilomatrixoma: Cytologic features on thinprep and diagnostic pitfalls

Dear Editor, Pilomatrixoma (Px) or calcifying epithelioma of Malherbe is composed of basaloid cells and squamoid cells with ghost nuclei (shadow cells) within a cellular stroma, calcium deposits, and multinucleated giant cells. Sampling certain components can lead to diagnostic misinterpretation. Moreover, given the rarity of cytologic specimens and widespread use of liquid-based preparations (LBP), awareness of the cytology of Px and diagnostic pitfalls is important. Herein, we compare cytomorphologic features of Px on ThinPrep [(TP), Hologic Inc. Boxborough, MA] with CS in a case series. Furthermore, we review the differential, diagnostic pitfalls, and ancillary testing for Px in cytologic specimens. A retrospective search of the New York Presbyterian-Weill Cornell Medicine archival pathology database from 2007 to 2016 yielded 113 histologic Px specimens. Six cases had pre-operative cytology (5.4%) and one had a concurrent preoperative biopsy. The mean age was 24.1 years (range 3 months–70 years) with a male:female ratio of 1.5:1. Fine-needle Aspiration (FNA) with a 25-gauge needle was processed as one air-dried Diff-Quik (DQ) stained and one alcohol-fixed Papanicolaou (Pap) stained CS. Residual specimen when collected in CytoLyt (Hologic Inc., Boxborough, Massachusetts) was processed as one Papstained TP and one hematoxylin and eosin (H&E)-stained cell block (CB) slide. Four cases had CS and TP, one had CS only and one had TP only. Four cases were diagnosed as Px, one case was diagnosed as an inflamed epidermal inclusion cyst and one case was diagnosed as “suspicious for squamous cell carcinoma (SCC).” Immunohistochemistry was performed on CB material with anti-b-catenin monoclonal antibody (14; BD 610154) as per manufacturer instructions. The DQ and Pap smears showed high cellularity with mostly ghost cells in syncytia, single cells and clusters with dense, orangeophilic

Fine needle aspiration of meningioma: Cytologic features on ThinPrep

Dear Editor, Meningiomas represent the most common primary brain tumors arising from the arachnoid villi, but can present in unusual locations. Recent studies have described the cytologic features of meningioma on conventional smears (CS), including whorling pattern, intranuclear grooves (INGs) and pseudoinclusions (INIs), and delicate cytoplasmic extensions. Herein, we report cytomorphologic features of an extracranial meningioma on ThinPrep [(TP), Hologic Inc. Boxborough, MA] and compare with CS, which, to our knowledge, have not been described. Liquid-based preparations (LBP), including TP and SurePath (SP) are increasingly being used for fine-needle aspiration (FNA) cytology. Extracranial meningiomas pose a diagnostic challenge and knowing the cytological characteristics in LBP will aid with the diagnosis. The patient is an 84-year-old woman who presented with a yearlong history of a firm facial lesion in the right sphenotemporal region. MRI of the face/orbit revealed a 6.5 3 4.2 3 3.6 cm T2 hypointense, homogenously-enhancing, diffusion restricting mass, centered on the right sphenotemporal buttress, involving the infratemporal fossa with direct intracranial extension into the lateral aspect of the middle cranial fossa and into the orbit (Figure 1A). With these imaging studies, the differential diagnosis included meningioma, lymphoma, metastasis, and hemangiopericytoma. A FNA of the mass was performed in three passes with a 25gauge needle. The specimen was processed as one air-dried Diff-Quik (DQ) stained and one alcohol-fixed Papanicolaou (Pap) stained CS. Residual specimen collected in CytoLyt (Hologic Inc. Boxborough, MA) was processed as one Pap-stained TP and one hematoxylin and eosin (H&E)-stained cell block (CB) slide. The DQ smear showed high cellularity with tumor cells in syncytial sheets and whorls (Figure 1B). Cells were predominantly elongate with abundant pale/eosinophilic cytoplasm and characteristic terminal wispy extensions with eccentric and pale nuclei. Pap stained slides showed

Mammary juvenile papillomatosis (“Swiss cheese” disease): Study of 121 cases reiterates need for long-term follow-up

Dear Editor, Juvenile papillomatosis (JP) is a rare, localized, and unilateral breast disease that typically effects younger women.1–6 The disease usually presents as a palpable, firm, circumscribed, and mobile mass. Imaging studies and gross examination show a mass with variably sized cysts. The latter nearly always span less than 2.0 cm. The multicystic appearance resembles Swiss cheese—which explains the alternate designation for JP, ie, “Swiss cheese” disease. Microscopically, JP exhibits changes including (a) ductal papillomatosis, (b) cysts: apocrine and non‐apocrine, (c) florid and papillary apocrine hyperplasia, (d) sclerosing adenosis, and (e) duct stasis with histiocytes.1–6 Rosen et al first described JP in 1980, and in the seminal paper announced the establishment of a registry for the disease. In 1982, based on data in this registry, Rosen et al concluded that JP “may be a marker for breast cancer for the patients family” and “the patient may be at increased risk.” In 1985, Rosen et al reported on a cohort of 180 JP cases, four of whom had concurrent invasive carcinoma (ipsilateral: 2, contralateral: 2) and three had classic type of lobular carcinoma in situ (LCIS) (all three ipsilateral). One patient developed “early” invasive ductal carcinoma 9 years later, and another developed ductal carcinoma in situ (DCIS) 12 years later— both patients had bilateral JP (synchronous in the former case, and metachronous in the latter). About 28% of patients had family history of breast carcinoma, mostly in second‐degree relatives. This report established the need for “careful clinical surveillance” of patients with JP and female relatives thereof. In 1990, Rosen et al published another study of 41 JP patients with a median follow‐up of 14 years, and concluded that the risk for carcinoma in these patients “should be greatest with a positive family history and recurrent bilateral JP.” The clinical implications of JP have not been further elucidated since these four reports by Rosen et al—except in much smaller series and in case reports. 6, 8–10 We reviewed 121 cases diagnosed with JP over a 17‐year period (2001‐2017). The pathological diagnosis was confirmed in each case. Key clinical and pathological data were obtained and analyzed. The mean age at diagnosis was 34.1 (range 13‐77) years. 120 patients were female. Four cases presented with bilateral masses. Of those that were unilateral, JP involved the left breast in 59, and the right in 58. 94 (78%) presented with a palpable mass, and 16 (13%) with an imaging abnormality (usually a mass with cysts). Grossly, each specimen was multicystic (Figure 1). Microscopically, all showed a variety of proliferative and nonproliferative changes (vide supra) (Figure 2). One (0.8%) case of classic invasive lobular carcinoma associated with lobular carcinoma in situ (LCIS) in a 46‐year‐old woman was concurrent at initial diagnosis of JP (and was intimately associated with the index lesion). Additionally, contemporaneous at initial diagnosis were four (3.3%) cases of ductal carcinoma in situ (DCIS). The DCIS cases occurred in women with a mean age of 47.5 (range 28‐

80 Cytological Features of Fine Needle Aspiration of Meningioma on ThinPrep

atypical glandular cells of endometrial origin in a woman ≥45 years old but ultimately reported as negative for malignant cells. Discussion: Collagen balls within peritoneal washings can be misinterpreted as fragments of atypical glandular cells, as was initially screened in our case, and also as papillary serous neoplasm or mucin distended cells exfoliated from a neoplasm. The crowded clusters of mesothelial cells could mimic hyperchromatic crowded groups of highgrade squamous intraepithelial lesion, leading to a misinterpretation. An awareness of this entity while screening cervical samples will help avoid such misdiagnosis.