Endoscopic ultrasound guided fine-needle aspiration diagnosis of duodenal high grade neuroendocrine carcinoma underlying a villous adenoma: report of a case

Endoscopic ultrasound guided fine-needle aspiration biopsy is a reliable and accurate method for the diagnosis of submucosal lesions of the gastrointestinal tract. We report the cytopathologic findings of a case of duodenal high-grade neuroendocrine carcinoma in a 68-year-old woman who presented with melena and marked anemia, 45 years after kidney transplantation. Imaging studies performed in the work-up of melena showed a duodenal mass, which on endoscopy proved to be an exophytic, villous duodenal lesion, 3 cm from the ampulla. Forceps biopsy of the exophytic lesion showed a villous adenoma. Endoscopic ultrasound additionally revealed an underlying submucosal lesion and EUS-guided fine needle aspiration of this submucosal mass and of the enlarged mesenteric lymph nodes was diagnostic of a high-grade neuroendocrine carcinoma. The aspirates showed abundant cellularity with tumor cells arranged in sheets and occasional loose clusters. The neoplastic cells had a moderate amount of pale cytoplasm and large round to oval hyperchromatic nuclei with focally prominent nucleoli. Mitoses, apoptotic bodies and necrotic debris were also present. The tumor cells were strongly and diffusely positive for cytokeratin AE1/AE3, synaptophysin and chromogranin and showed a very high proliferative fraction on Ki67 staining, supporting the diagnosis of a high-grade neuroendocrine carcinoma. This is to our knowledge the first case of high-grade neuroendocrine carcinoma of the duodenum diagnosed by EUS-FNA. This case also emphasizes the diagnostic value of EUS-FNA sampling of the submucosal and intramural component of villous tumors of the gastrointestinal tract when mucosal forceps biopsies show only benign findings.     

Aspiration cytopathology of middle-ear neuroendocrine carcinoma

Primary neuroendocrine carcinoma (carcinoid tumor) is among the rarest of middle-ear (ME) neoplasms, with only a rare case of metastatic disease having been reported. Fine-needle aspiration biopsy (FNAB) was performed in a 51-yr-old male with a two-decade history of multiple local recurrences from a right middle-ear neoplasm, with the most recent surgical excision 2 yr ago. He currently presented with an enlarged right parotid gland, and a right infratemporal mass. Aspirate smears showed a monotonous population of cytologically bland cells with a small to moderate amount of pale granular cytoplasm, round to oval nuclei, inconspicuous nucleoli, and finely granular chromatin. Rare, isolated large cells were occasionally seen. Immunohistochemical staining of the cell block made from the aspirated material showed strong cytoplasmic positivity for chromogranin, synaptophysin, neuron-specific enolase (NSE), serotonin, and cytokeratin cocktail, and negative staining for S100 protein. Review of tissue slides from the patient's prior middle-ear tumor showed an identical immunoprofile and morphology, and led to a revision of the original diagnosis of paraganglioma. Middle-ear neuroendocrine carcinoma has a low but definite metastatic potential, which can be diagnosed using FNAB if ancillary immunohistochemical studies are available.     

Peripancreatic paraganglioma mimics pancreatic/gastrointestinal neuroendocrine tumor on fine needle aspiration: Report of two cases and review of the literature

Cytologic diagnosis of extra‐adrenal paraganglioma presenting as a peripancreatic mass is challenging with a high error rate due to its rarity. We report two cases of peripancreatic masses identified by radiology. Endoscopic ultrasound‐guided fine needle aspiration (FNA) of the masses showed a moderately cellular tumor composed of small to medium sized neoplastic cells with round to oval nuclei, arranged singly and in loose clusters. Focal rosette‐like structures were present. The cells were positive for neuroendocrine markers (synaptophysin and chromogranin). A diagnosis of a neoplasm with neuroendocrine differentiation and neuroendocrine tumor was made respectively on FNA for each case. The subsequent surgical resection of the tumors revealed peripancreatic paraganglioma. Although paraganglioma has been reported in the literature, the detailed comparison of perpancreatic paraganglioma versus pancreatic/gastrointestinal neuroendocrine tumor is still lacking. Therefore using these two cases with literature review, we wish to illustrate the differential diagnosis between these two entities based on cytomorphology and immunohistochemical study.     

Fine-needle aspiration biopsy of a glomus tumor of the stomach

A glomus tumor of the stomach was found as an incidental finding on routine ultrasound in a 72-yr-old asymptomatic woman. A fine-needle aspiration biopsy (FNAB) was performed and was initially interpreted as a well-differentiated neuroendocrine neoplasm, possibly a carcinoid tumor. The aspirate revealed tightly packed nests or clusters of uniform, small, round to polygonal cells with scanty, faintly eosinophilic or clear cytoplasm and ill-defined cell borders. The nuclei were uniform, and round to oval, and contained a granular chromatin pattern and inconspicuous nucleoli. Very occasional intranuclear cytoplasmic inclusions were seen. Laparotomy and a wedge resection of the stomach were performed. The surgical pathology findings revealed a glomus tumor which was confirmed by immunohistochemical stains and ultrastructural studies. Since glomus tumors of the stomach are essentially benign and are amenable to conservative excision, it is important to separate them, preoperatively, from more aggressive gastric neoplasms. FNAB offers a rapid, cost-effective method of diagnosing this entity. We present the cytological, histological, ultrastructural, and immunocytochemical features of this particular gastric neoplasm, along with differential diagnoses.     

Lipid-rich carcinoid tumor of the thymus gland: diagnosis by fine-needle aspiration biopsy

In this report we describe the cytologic features of an unusual thymic carcinoid tumor containing prominent cytoplasmic lipid vacuoles that was diagnosed by fine-needle aspiration biopsy. The tumor presented as a presternal subcutaneous mass in an 81-yr-old female, with a contiguous mediastinal mass on computed tomography. The cytomorphologic features included numerous discohesive cells with eccentric, round to oval nuclei, granular chromatin, and scant cytoplasm containing numerous intracytoplasmic, clear vacuoles. The neoplastic cells were reactive for neuroendocrine markers by immunocytochemistry and showed reactivity of the intracytoplasmic vacuoles with an oil red-O stain for neutral lipid. Ultrastructural examination confirmed the presence of intracytoplasmic lipid vacuoles and neurosecretory granules. Subsequent surgical excision confirmed the diagnosis. We believe this to be the first report describing these features in a primary thymic carcinoid tumor. The differential diagnosis of mediastinal tumors with clear-cell features and cytoplasmic vacuolization is also discussed.     

Fine-needle aspiration cytology of metastatic carcinoid tumor: report of a case and review of the literature

While carcinoid tumor is a relatively common neoplasm in surgical pathology, fine-needle aspiration (FNA) cytology as a method of primary diagnosis has only been reported in the literature a few times. We report on the case of a 42-yr-old female with multiple large metastatic tumor deposits in her liver, pelvic adnexae, bones, and lungs, with an unknown primary. FNA was performed on one of the liver masses, and was diagnosed using routine histochemical and immunohistochemical stains as carcinoid tumor. No follow-up tissue diagnosis has been obtained. However, the patient is still alive with her tumor 1 yr later. Fine-needle aspiration cytology can be a useful and safe tool in the diagnosis of metastatic carcinoid tumors, avoiding the need for surgery which would not otherwise be indicated for treatment.     

Diagnosis of metastatic carcinoid in mesenteric lymph node by fine needle aspiration cytology

Although carcinoid tumor is a relatively common neoplasm in surgical pathology, fine needle aspiration cytology (FNAC) as a method of primary diagnosis has only been reported a few times. We report the case of a 55-year-old male patient who presented with colicky pain in the abdomen and a vague mass in the right lumbar region. Ultrasonographic study showed an enlarged mesenteric lymph node. Ultrasonography-guided FNAC revealed cellular smears with neuroendocrine cellular arrangement and morphology. The cytomorphologic characteristics of carcinoid tumors are distinctive enough for diagnosis. A diagnosis of metastatic carcinoid was given. 5-hydroxyindolacetic acid in 24-hour urine turned out to be high. Histopathology and immunohistochemistry studies also confirmed the diagnosis. Thus, FNAC can be a useful and safe tool in the diagnosis of carcinoid tumors.     

Hepatoblastoma in a 15-month-old male: cytomorphology, electron microscopy, and differential diagnosis

The authors describe a case of hepatoblastoma in a 15-month-old male and discuss the differential diagnosis and electron microscopic features of small round cell tumors. The patient was found to have an enlarged liver and was admitted to the hospital for further investigation. Fine-needle aspiration of the liver revealed small, uniform cells with increased nuclear/cytoplasmic ratio and focal rosette formation. A diagnosis of small blue cell neoplasm favoring hepatoblastoma was made, but neuroblastoma could not be ruled out. Electron microscopic analysis performed on the liver aspirate showed features of hepatic differentiation as well as absence of neuroblastic differentiation. The diagnosis of hepatoblastoma was made. Serum alpha-fetoprotein level of 33,250 mg/L confirmed the diagnosis. Liver biopsy performed subsequently showed tumor cells arranged in nests, acini, and trabeculae with mitotic figures. Electron microscopy showed the same findings as described above. The patient underwent chemotherapy for 4 months and subsequently a partial liver resection was performed. This case illustrates the important role of electron microscopy in evaluating small round cell tumors in children.     

Pulmonary carcinoid with papillary structure: Report of a case with fine-needle aspiration cytology

Fine-needle aspiration biopsy of a peripheral lung mass in a 44-yr-old man revealed a papillary tumor. Histologic examination of the mass, removed by lobectomy, demonstrated a pulmonary carcinoid tumor with papillary structure. Most papillary tumors of the lung are malignant. Pulmonary carcinoid and pulmonary sclerosing hemangioma—benign tumors—have papillary variants. Both have been described in fine-needle aspiration specimens and should be included in the differential diagnosis of papillary lung tumors. The cytologic features of pulmonary carcinoid are described. Diagn Cytopathol 1994;11:178–181. © 1994 Wiley-Liss, Inc.     

Bronchial carcinoid tumor with amyloid stroma

The histopathologic, immunohistochemical, and ultrastructural findings of a unique case of an atypical bronchial carcinoid tumor containing abundant stromal amyloid are presented. The differential diagnosis from other neuroendocrine tumors that can produce amyloid is discussed.     

Myoepithelioma of soft tissue: A cytological-pathological correlation with literature review

Myoepitheliomas of soft tissue are rare tumors with variable morphologic, immunohistochemical and molecular profiles and therefore are diagnostically challenging for pathologists. We report a case in a 60-year-old male with a painless slowly growing 3 cm mass on left medial forefoot. Core biopsy of the mass showed a neoplastic proliferation of plasmacytoid tumor cells, consistent with myoepithelioma of soft tissue. Immunohistochemical stains demonstrated positivity of the tumor cells for cytokeratin AE1/AE3, CK18, S-100 protein and myosin heavy chain (SMMS-1), supporting the diagnosis. Fine-needle aspiration was performed intraoperatively before the resection of the mass. Air-dried Diff-Quik stained cytology slides showed singly scattered and loosely cohesive clusters of plasmacytoid and spindle cells with dense basophilic cytoplasm, distinct cytoplasmic borders, and round to oval mildly pleomorphic nuclei with smooth nuclear membrane. Scattered naked nuclei, binucleated tumor cells, as well as tumor cells with wispy elongated cytoplasm were also seen. Occasional clusters of cells were intimately associated with metachromatic fibrillary stromal material. Histologic examination of the resected tumor confirmed the diagnosis of myoepithelioma. Molecular studies showed absence of EWSR1 rearrangement. Cytological differential diagnosis, clinical, histological, immunohistochemical, and molecular features of soft tissue myoepitheliomas are discussed in this study.     

Fine-needle aspiration biopsy diagnosis of extrarenal malignant rhabdoid tumor

Two cases of extrarenal malignant rhabdoid tumors are presented in which diagnosis was suggested by fine-needle aspiration biopsy and confirmed by histologic and electron microscopic examination. Fine-needle aspiration smears in both cases revealed round to polygonal cells with vesicular nuclei and prominent nucleoli. Several tumor cells contained cytoplasmic inclusions composed of intermediate filaments. A majority of the tumor cells stained strongly for vimentin and cytokeratin. Electron microscopic examination revealed many cells with large aggregates of intermediate filaments corresponding to the cytoplasmic inclusions. Fine-needle aspiration biopsy may be used for diagnosing malignant rhabdoid tumor. The diagnosis may be further confirmed by immunohistochemistry and electron microscopy. Diagn Cytopathol 1994;11:271–276. © 1994 Wiley-Liss, Inc.     

Low-grade endometrial stromal sarcoma presenting as multiple pulmonary nodules: A potential pitfall in fine needle aspiration and core biopsy specimens - A Cytological - Pathological Correlation

Low-grade endometrial stromal sarcoma (LGESS) is the second most common malignant mesenchymal tumor of the uterus. The most common location is the uterine corpus, but it can also primarily arise in a variety of extrauterine locations such as pelvis, ovary, abdominal cavity, vagina, and vulva. We are reporting a case of a 47-year-old female with no significant medical history who presented with multiple pulmonary nodules. Fine needle aspiration (FNA) specimen revealed spindle cell neoplasm consistent with the diagnosis of LGESS. The differential diagnosis included neuroendocrine tumor, synovial sarcoma, solitary fibrous tumor, smooth muscle tumors, and peripheral nerve sheath tumors. The clinical, cytological, and histopathologic details of this case, as well as a discussion of the potential pitfalls and differential diagnosis of spindle cell lesions of the lung are described.     

A metastatic renal carcinoid tumor presenting as breast mass: a diagnostic dilemma

We present clinicopathological and cytological findings of a well-defined breast mass in a patient with history of primary renal carcinoid tumor. Fine-needle aspiration (FNA) cytology showed monotonous tumor cells with plasmacytoid appearance arranged singly and in small clusters. Occasional tumor cells were arranged in acinar architecture resembling glandular differentiation. Tumor cells showed fine speckled chromatin. The unusual location for metastasis of this rare type of carcinoid tumor and overlapping cytological features with primary mammary carcinoma led to an erroneous preliminary cytological diagnosis of primary breast carcinoma with plasmacytoid features. Tumor cells in the corresponding cell block showed strong diffuse positivity for synapthophysin and pan-cytokeratin with weak focal positivity for chromogranin markers. These patterns of immunostaining were similar to the original renal carcinoid tumor. To the best of our knowledge, a few cases of carcinoid tumor metastatic to the breast have been reported in the literature and more than half of these cases were initially misdiagnosed as primary breast carcinoma causing unnecessary surgical treatment. This is a first reported case of metastatic renal carcinoid tumor into breast diagnosed with FNA biopsy. This report highlights the cytological features of well-differentiated neuroendocrine tumor (carcinoid tumor) and its potential diagnostic pitfalls.     

Aspiration cytology of papillary cystic neoplasm of the pancreas

The authors present a case of papillary and cystic neoplasm of the pancreas (PCN) in which fine-needle aspiration was performed intraoperatively. Only a few reports of fine-needle aspiration of this rare tumor have been published. The features most helpful in reaching a diagnosis of PCN were a monotonous population of tumor cells, round to oval bland-appearing nuclei, scanty to moderate ill-defined cytoplasm, and the presence of numerous capillaries. Despite the lack of branching papillary clusters described in previous reports, it was possible to suggest the correct diagnosis by identifying these other characteristic features. In addition, estrogen and progesterone receptor levels were measured and found to be negligible. A review of the literature with emphasis on cytologic features also is presented.     

Hepatoblastoma in a 15-Month-Old Male: Cytomorphology,Electron Microscopy,and Differential Diagnosis

The authors describe a case of hepatoblastoma in a 15-month-old male and discuss the differential diagnosis and electron microscopic features of small round cell tumors. The patient was found to have an enlarged liver and was admitted to the hospital for further investigation. Fine-needle aspiration of the liver revealed small, uniform cells with increased nuclear/cytoplasmic ratio and focal rosette formation. A diagnosis of small blue cell neoplasm favoring hepatoblastoma was made, but neuroblastoma could not be ruled out. Electron microscopic analysis performed on the liver aspirate showed features of hepatic differentiation as well as absence of neuroblastic differentiation. The diagnosis of hepatoblastoma was made. Serum alpha-fetoprotein level of 33,250 mg/L confirmed the diagnosis. Liver biopsy performed subsequently showed tumor cells arranged in nests, acini, and trabeculae with mitotic figures. Electron microscopy showed the same findings as described above. The patient underwent chemotherapy for 4 months and subsequently a partial liver resection was performed. This case illustrates the important role of electron microscopy in evaluating small round cell tumors in children.     

Ossifying fibromyxoid tumor: Report of a case with cytomorphologic description

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm with uncertain histogenesis. Most cases behave in a clinically benign fashion; however, a small percentage of tumors may locally recur or metastasize. Herein we present a case of a 56‐year‐old man who presented with an enlarging left groin mass, left inner thigh numbness, burning paresthesia and discomfort in his left groin. The mass sampled by fine‐ needle aspiration and needle core biopsy. Cytology showed bland‐appearing epithelioid cells with round nuclei and fine chromatin, with fragments of fibromyxoid stroma in the background. Immunohistochemical stains performed on the core biopsy showed that the lesional cells were focally positive for S100 protein and negative for desmin, smooth muscle actin, CD34 and cytokeratin AE1/AE3. A benign neoplasm was favored with ossifying fibromyxoid tumor as the main entity in the differential diagnosis. A subsequent resection showed a well‐circumscribed 5 cm mass with firm consistency and focal areas of calcifications. Histologically, the tumor had a nodular growth pattern with relatively bland spindle cells containing round to oval nuclei suspended in a variably collagenous to myxoid stroma. Significant ossification and bone formation was also noted. There was no significant atypia, necrosis or increased mitoses. Ossifying fibromyxoid tumors have distinct cytologic features and should be considered in the differential diagnosis of soft tissue tumors with prominent ossification. Diagn. Cytopathol. 2015;43:646–649. © 2015 Wiley Periodicals, Inc.     

Interdigitating dendritic reticulum cell sarcoma: cytologic, histologic and immunocytochemical features

Tumors of dendritic reticulum cells are rare neoplasms that exhibit significant morphologic overlap with other malignancies. Fine-needle aspiration cytologic appearances of this neoplasm are not well understood. A 33-yr-old woman presented with a rapidly growing nodular mass in the right upper cervical region and right-sided ptosis. Fine-needle aspiration cytology of the mass showed dissociated as well as clustered, large, polygonal cells that showed high nuclear-cytoplasmic ratio. Nuclei were round, oval, or irregular in shape. Large and small blastoid forms with prominent nucleoli and chromatin clumping as well as binucleated cells and cells with lobulated nuclei were seen. Numerous mitoses were observed. The tumor cells expressed focal immunocytochemical reactivity to CD45 and CD68, but were negative for CD2, CD3, CD4, CD8, CD20, CD30, CD45RO, epithelial membrane antigen (EMA), cytokeratin, and HMB45. Histologic sections of the biopsy from the growth showed nodal tissue effaced by a tumor composed of large, pleomorphic neoplastic cells with some binucleate and multinucleate forms resembling Reed-Sternberg cells. The intervening stroma contained numerous small lymphocytes. Tumor cells expressed vimentin, S-100 protein, CD68, and MAC387, but were negative for LCA, CD1a, CD3, CD15, CD20, CD21, CD23, CD30, CD35, carcino-embryonic antigen, HMB45, cytokeratin AE1/3, EMA, myeloperoxidase, lysozyme, smooth-muscle actin, and desmin. The combined histologic and immunohistologic features suggested a histiocytic/dendritic reticulum cell neoplasm and a diagnosis of interdigitating dendritic reticulum cell sarcoma was made.     

Aspiration biopsy of granulosa-cell tumor of the ovary: cytologic findings and differential diagnosis.

Fine-needle aspirates from four histologically confirmed adult granulosa-cell tumors of the ovary revealed irregular sheets, loose aggregates or tight clusters of small tumor cells with round or oval nuclei and scant cytoplasm. Nuclear grooves and indentations were noted in a small number of cells in two cases. Tumor cells arranged in follicular pattern mimicking Call-Exner bodies were not identified in all cases. A cytologic differential diagnosis of "small-cell" tumors of the ovary is briefly discussed.