Search

Clear cell Mullerian-type adenocarcinoma of the testis is an exceedingly rare entity, and its histogenesis and clinical behavior are still poorly understood. We discuss three cases of clear cell carcinoma of the testis, compiled from a review of the literature and our personal experience. Microscopically, the tumors closely resembled clear cell carcinoma of the ovary, displaying papillae lined by clear cells with areas of hobnailing. The reported immunophenotypic features were also similar to that of ovarian tumors, as positivity for epithelial markers (CK7, CAM5.2, AE1/AE3, EMA) and Mullerian markers (PAX8, CA125) with negativity for estrogen and progesterone receptors have been observed. The pathogenesis of testicular clear cell carcinoma is still poorly understood, with reported cases displaying evidence of both mesothelial and Mullerian origin. In addition, molecular characterization of testicular clear cell carcinomas has yet to be accomplished; however, studies performed on ovarian clear cell carcinomas may provide insight to the origin, biologic behavior, and potential therapeutic modalities for this obscure, aggressive malignancy. • Clear cell tumors in ovary and testis share similar histology and immunoprofile. • Pathogenesis of testicular clear cell tumors may be Mullerian or mesothelial. • Molecular features of ovarian clear cell tumors may guide future studies in testis. [ABSTRACT FROM AUTHOR]

Abstract Intraluminal crystalloids have rarely been described in the breast, particularly in cases with ductal carcinoma in situ (DCIS). We recently encountered a case of DCIS of the breast associated with numerous intraluminal crystalloids. The patient presented with a mass in the right breast, and microcalcifications were detected on screening and diagnostic mammograms; the patient underwent needle biopsies, lumpectomy, and skin-sparing mastectomy. Invasive ductal carcinoma associated with extensive DCIS was diagnosed. Multiple refractile, eosinophilic crystalloids, with variable morphologies including rectangular, triangular and needle-like with sharp borders, were observed within the lumina of DCIS, besides calcium phosphate microcalcifications. We report this case together with a literature review on crystalloid-containing lesions in breast and non-breast tissues. We also studied radiologic findings of these crystalloids using a specimen radiograph. Highlights • Intraluminal crystalloids are refractile, eosinophilic structures with variable morphologies. • The morphology of intraluminal crystalloids of breast resembles those of prostate, salivary glands, and struma ovarii. • Intraluminal crystalloids are a rare pathologic finding and diagnostic significance is controversial. • Careful search for cancer is recommended when cancer is not initially detected in a case with intraluminal crystalloids. • Intraluminal crystalloids are not detected on radiologic evaluation. [ABSTRACT FROM AUTHOR]

Sarcoid-like (SL) granulomas have been previously described in association with malignant tumors. These granulomas appear to be tumor-related but are not indicative of systemic sarcoidosis, and hence are referred to as SL reactions. These SL reactions can be seen within the primary tumor, its vicinity, or in uninvolved sites such as the spleen, bone marrow, skin, and/or regional lymph nodes draining the tumor. It is a widely held view that SL granulomas are caused by soluble antigenic factors, shed by tumor cells or released due to tumor necrosis. SL reactions reported in Hodgkin lymphoma have been associated with a better prognosis. SL granulomas are thought to play an important role in the host's defenses against metastatic extension. SL granulomas have been reported in approximately 4.4% of carcinomas. Isolated cases of renal cell carcinoma (RCC) with SL granulomas have been reported with questionable prognostic significance. We identified 11 cases of RCCs with SL granulomas. Interestingly, all cases had abundant clear cell cytoplasm (10 clear cell RCC cases and 1 clear cell papillary RCC). We propose that this clear, abundant cytoplasm of the tumor cells with high content of glycogen and lipids may trigger granuloma formation akin to that seen in seminomas with SL granulomas. To date, this is the largest case series of RCCs with SL granulomas. [ABSTRACT FROM AUTHOR]

Background There are well known cases of hybrid tumors of chromophobe renal cell carcinoma (RCC) and oncocytoma in kidney, where both tumors have the same cell of origin – intercalated cell of the collecting duct. However, collision tumors composed of neoplasms originating from different cell lineages such as oncocytoma and papillary RCC are extremely rare. Herein, we made a collective literature review of reported cases of collision tumors composed of oncocytoma and papillary RCC, adding a case that we recently experienced. Material and methods A PubMed database was search for collision tumors of the kidney composed of oncocytoma and papillary RCC and a collective literature review was made. To this cohort, we also added a recently encountered case with similar, confirmed by immunohistochemistry, morphological features. Results To date 8 cases of a collision tumor composed of papillary RCC and oncocytoma have been described in the literature. All of them had a smaller papillary RCC component present within a larger oncocytoma. Conclusion Because of a few cases of such a collision tumors reported, it is difficult to make classification and right clinical management of these patients. None of the reported cases had tumor recurrence or progression on a follow-up. The presence of only small portion of papillary RCC in a large oncocytoma raises a possibility of under-sampling of malignant component in large oncocytomas in core biopsy or surgically resected specimens. We recommend better sampling, particularly at the periphery of otherwise classic oncocytomas to unveil this possible association. [ABSTRACT FROM AUTHOR]

Inflammatory myofibroblastic tumor (IMT) is an uncommon lesion that shows a wide range of anatomic distribution. The adrenal gland, however, is a distinctly rare site of occurrence. To date, only a few cases of IMT arising in the adrenal gland have been reported in the English literature. Here, we report another case of isolated adrenal IMT. A 34-year-old man presented to the emergency department with a complaint of a sudden severe right-sided back pain. Subsequent computed tomographic scan imaging studies demonstrated a large right adrenal mass associated with a hematoma. The right adrenal gland was resected. Microscopic examination revealed an encapsulated cellular spindle cell proliferation with a prominent inflammatory infiltrate. Immunohistochemically, those spindle cells were diffusely and strongly positive for anaplastic lymphoma kinase-1, and focally and weakly positive for smooth muscle actin. S-100 protein and cytokeratin were negative. The findings were consistent with IMT arising from the adrenal gland. Although IMTs in the adrenal gland are rare, they should be considered in the differential diagnosis of adrenal masses. The clinical behavior of IMTs in general is currently indeterminate and a close clinical follow-up is recommended. The behavior of adrenal IMTs remains uncertain because of rare reported cases and lack of long-term follow-up. Further follow-up of reported cases and recognition of additional new cases is warranted to unmask the true biological behavior of adrenal IMTs. [ABSTRACT FROM AUTHOR]

Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently described rare mesenchymal tumor of borderline malignancy. It generally involves superficial soft tissue, with a predilection to the lower extremities. Microscopically this tumor is characterized by a fascicular and storiform growth pattern, spindled to epithelioid cells, nuclear atypia with pleomorphism, and eosinophilic granular, and fibrillar to glassy cytoplasm. Strong diffuse immunoreactivity for CD34 is very characteristic of this entity. Due to under-recognition, this tumor is generally underreported. Additionally, cases of recurrence are rarely reported in the literature. We will comprehensively review the English language literature on all reported cases of SCPFT, with emphasis on recurrence. • Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently described rare mesenchymal tumor of borderline malignancy. • SCPFT generally involves superficial soft tissue, with a predilection for lower extremities. • Microscopically, SCPFT is characterized by marked nuclear pleomorphism, low mitosis, and diffuse CD34 positivity. • This tumor is usually underrecognized and underreported. Additionally, cases of recurrence are rarely reported. • This paper provides a comprehensive review of SCPFT cases in the literature with a focus on cases of recurrence. [ABSTRACT FROM AUTHOR]

Clear cell papillary renal cell carcinoma (CCP-RCC) has recently been recognized as a distinct subtype of renal cell carcinoma (RCC) due to its unique morphologic, immunohistochemical, and genetic features and indolent clinical behavior. However, the incidence of this tumor in a nephrectomy series for renal mass has not been fully investigated. Twelve cases of CCP-RCC were identified from a total of 290 consecutive partial (n = 137) or radical nephrectomies (n = 153) for RCC from 2010 to 2012 in our hospital. In this series, CCP-RCC was the fourth most common (4.1%) kidney tumor following clear cell (conventional) (70%), papillary (16.6%), and chromophobe (5.9%) RCCs. The average age of the CCPRCC patients was 58.2 years (range, 18-81 years), with an equal sex distribution. Four cases (33.3%) were associated with end-stage renal disease. Of the 12 CCP-RCCs, 9 presented as solitary tumors; 2 coexisted with clear cell RCC; and 1 with papillary RCC. The average size of tumors was 2.5 cm (range, 0.8-6.0 cm). All tumors were pT1 (10 pT1a and 2 pT1b). Two cases were initially misclassified as clear cell RCC. Strong positive cytokeratin 7 stain and negative stains with a-methylacyl-CoA racemase and RCC marker differentiate CCP-RCC from low-grade clear cell RCC with similar histologic features. We conclude that CCP-RCC is a common renal neoplastic entity, representing the fourth most common (4.1%) RCC. It can be easily misclassified due to its overlapping features with low-grade clear cell RCC. In equivocal cases, immunohistochemical stains with a small panel of markers (cytokeratin 7, α-methylacyl-CoA racemase, RCC marker, or CD10) are warranted in making the correct histologic classification. [ABSTRACT FROM AUTHOR]

Abstract: Solid-pseudopapillary neoplasm (SPN) of the pancreas is a rare neoplasm that most commonly affects adolescent girls and young women. Solid-pseudopapillary neoplasm of the pancreas is considered to have malignant potential; 10% to 15% of cases are associated with metastasis, and these usually present at the time of initial diagnoses. Cases with metastases after resection are rare, and all reported cases have occurred less than 5 years after resection. We report a case of SPN in a 36-year-old woman who presented with liver metastasis 15.8 years after complete resection of the primary tumor in the pancreas. To the best of our knowledge, this is the longest time interval reported between resection of primary tumor and subsequent detection of metastatic disease. Solid-pseudopapillary neoplasm of the pancreas can present with metastases many years after resection of the primary tumor. Long-term follow-up is warranted, given the possibility of late metastasis. [Copyright &y& Elsevier]

Abstract: Primary cardiac angiosarcoma is a rare but the most common malignant neoplasm of the heart in adults. The objective of this study is to analyze the clinicopathologic characteristics of primary cardiac angiosarcoma. Ten cases of primary cardiac angiosarcoma treated in a single institution were analyzed for their clinical, pathologic, and immunohistochemical features. There were 6 men and 4 women, with a mean age of 40 years (range, 20-61 years). The patients commonly presented with dyspnea and distant metastasis. All tumors were located in the right atrium, with a mean tumor size of 6.8 cm. Tumors were hemorrhagic, with variegated tan-brown solid areas. Histologically, they exhibited high-grade morphology with mixed solid growth and anatomizing channels. Frequent mitoses and tumor necrosis were common. The tumors were strongly positive for CD31, CD34, FLI-1, and WT-1 but negative for AE1/3, D2-40, human herpesvirus 8, and epidermal growth factor receptor. The tumor cells were focally reactive to p53, with a high rate of Ki-67 expression. A complete tumor resection was not possible in any of the patients because of the size or extensive local invasion of the tumor. Overall survival ranged from 1 to 81 months (mean, 26.6 months) after initial histologic diagnosis. Primary cardiac angiosarcomas are rare tumors that commonly arise in the right atrium. The mean age is much younger than that of soft tissue angiosarcoma. Regional tumor extension and distant metastasis are extremely common at the time of diagnosis. Surgical resection with adjuvant chemotherapy is currently the preferred treatment, and survival time appears to be inversely correlated with the tumor size and degree of regional tumor extension at the time of surgery. [Copyright &y& Elsevier]

Summary: Recent progress in skeletal molecular biology has led to the clarification of the transcriptional mechanisms of chondroblastic and osteoblastic lineage differentiation. Three master transcription factors—Sox9, Runx2, and Osterix—were shown to play an essential role in determining the skeletal progenitor cells'' fate. The present study evaluates the expression of these factors in 4 types of benign bone tumors—chondromyxoid fibroma, chondroblastoma, osteoid osteoma, and osteoblastoma—using immunohistochemistry and tissue microarrays. Osteoid osteoma and osteoblastoma showed strong nuclear expression of Osterix and Runx2. In contrast, only a few chondroblastomas showed positive nuclear expression of Osterix. Strong nuclear expression of Sox9 was detected in all chondroblastomas, whereas nearly half of the osteoblastomas showed focal weak cytoplasmic expression of Sox9. [Copyright &y& Elsevier]

Abstract: The muscle layer in the cystic duct and common bile duct is not well defined, and it is unresolved whether it represents muscularis mucosae or muscularis propria. Smoothelin is a novel smooth muscle–specific contractile protein expressed only in fully differentiated smooth muscle cells of the muscularis propria and not in proliferative or noncontractile smooth muscle cells of the muscularis mucosae. In this study, we characterize the histologic aspects of the muscle layer in gallbladder, cystic duct, and common bile duct by evaluation of routine histologic sections and the utilization of immunohistochemistry using desmin and smoothelin. Formalin-fixed, paraffin-embedded sections of the gallbladder (15 cases), cystic duct (11 cases), and common bile duct (10 cases) were stained for smoothelin and desmin. Staining intensity was evaluated as weak or strong. The staining pattern score was evaluated as follows: 0 or negative = less than or equal to 5% positivity, +1 or focal = 6% to 10% positivity, +2 or moderate = 11% to 50% positivity, and +3 = greater than 50% muscle cells positivity. With desmin, strong and diffuse (+3) staining was observed in all gallbladder cases (15/15, 100%), highlighting one continuous muscle layer. The muscle layer was discontinuous and interrupted in all cystic duct cases and in most common bile ducts, highlighted by the desmin stain. Smoothelin intensely stained (at least +2) muscle fibers in the gallbladder in 11 (73%) of 15 cases similar to that observed with desmin staining. In contrast, common bile ducts predominantly had absent or weak and focal immunostaining (0 or +1 staining) with smoothelin (7/10, 70%), with only a few cases (3/10, 30%) having +2 staining (no cases with +3). Cystic ducts also showed absent or weak and focal immunostaining with smoothelin, with 5 (44%) of 11 cases showing 2+ immunostaining with smoothelin (no cases with 3+). Based on our findings, we conclude that, in the gallbladder wall, the muscle layer is muscularis propria and there is no muscularis mucosae present. In the cystic duct and common bile duct, only an attenuated and incomplete muscle layer of muscularis mucosae is present; because there is no muscularis propria, there probably is limited contractile function. Differentiating these anatomical muscle structures may be important for the pathologic staging of carcinoma in these organs. [Copyright &y& Elsevier]

Abstract: Because thyrolipoma (adenolipoma of thyroid) and thyrolipomatosis (diffuse lipomatosis of thyroid) are distinctively rare conditions with only few cases reported in the literature, we are reporting 5 additional cases. All the 5 patients were adult females, with ages from 38 to 79 years, who presented with thyroid masses. Four of the patients had normal thyroid function tests and one had mild hypothyroidism. All patients received partial or total thyroidectomy. The thyroid specimens showed either circumscribed yellow-tan masses (cases 1, 2, and 3) or diffuse yellow-brown discoloration (cases 4 and 5). Histologic examination revealed abundant mature fat infiltrating the affected thyroid tissue in 3 distinct patterns: (1) fat infiltration limited to follicular adenomas (thyrolipoma); (2) fat diffusely infiltrating throughout the thyroid gland (thyrolipomatosis); or (3) fat infiltration involving both follicular adenoma and their surrounding thyroid tissue. Because of the rarity of thyroid fat-containing lesions, confusion in differential diagnosis may occasionally occur. It is important to be aware during frozen section that these lesions may present as extrathyroidal nodules, which can be radioactive on intraoperative scan for parathyroid glands. In addition, a papillary thyroid carcinoma was also identified in one case of thyrolipomatosis. All patients recovered well after surgery and there has been no recurrence of the lesions after 1 to 24 years of clinical follow-up. In summary, we are reporting 5 rare cases of thyrolipoma and thyrolipomatosis with distinct histologic patterns. Previously reported cases of thyrolipomatosis were reviewed and analyzed with the current cases. [Copyright &y& Elsevier]

Abstract: Dedifferentiated liposarcoma with peculiar meningothelial-like whorling pattern and metaplastic bone formation (DDLMB) is an unusual morphologic entity that is characterized by an atypical lipomatous tumor/well-differentiated liposarcoma with epithelioid or spindle cells concentrically arranged into meningothelial-like “whorls,” and mature bone trabeculae rimmed by reactive osteoblasts. We recently experienced 2 cases of DDLMB, one in a 64-year–old male patient with a painless right groin mass and another in a 42-year–old female patient with a painless right abdominal mass. The size of the tumors was 3.5 and 18 cm; and the tumors were located in the right scrotal sac and retroperitoneum in case 1 and case 2, respectively. Under the initial clinical diagnosis of cord lipoma in case 1 and high-grade sarcoma in case 2, the masses were removed. The cut surfaces of the masses were well circumscribed with encapsulation, red-tan, firm, and multinodular. Microscopically, the tumors consisted of atypical lipomatous tumor/well-differentiated liposarcoma with meningothelial-like whorls and metaplastic bone formation in both cases. In addition, the first case showed focal areas of paraganglioma-like pattern; and the second case showed pleomorphic high-grade sarcoma with low-grade myxofibrosarcoma-like areas. Immunohistochemically, the tumor components with meningothelial-like pattern and paraganglioma-like pattern in DDLMB were positive for vimentin and CD56 and negative for pancytokeratin, epithelial membrane antigen (EMA), desmin, and smooth muscle actin. Characteristically, the paraganglioma-like area was immunoreactive for S-100 protein, with a “dot-like” staining pattern. The patient additionally underwent radical orchiectomy in case 1. Review of the literature revealed that only 34 cases of DDLMB have been reported. One case of dedifferentiated liposarcoma with a predominant paraganglioma-like pattern has also been reported in the literature. To our knowledge, case 1 represents the first report of DDLMB with paraganglioma-like pattern. A brief literature review was made with focus on the morphologic variations of DDLMB. [Copyright &y& Elsevier]

Summary: Prostatic adenocarcinoma is the most frequently diagnosed cancer in American men. Tumor Gleason grade and stage provide extremely valuable prognostic information and play an important role in therapeutic decision making and patient counseling. A biopsy or radical prostatectomy specimen revealing carcinoma extending into extraprostatic tissue permits a T3 classification. This is most easily recognized, particularly in a needle biopsy, when tumor is seen to invade the adipose tissue. The existence of intraprostatic adipose tissue is somewhat controversial. To investigate this, formalin-fixed paraffin-embedded whole-mount radical prostatectomy specimens from 427 patients with adenocarcinoma were evaluated for intraprostatic adipose tissue. It was defined as any collection of adipocytes amid or internal to the most peripheral glands. The amount, anatomic location, and relationship to normal structures were also recorded. Intraprostatic adipose tissue was identified in 17 (3.98%) of cases. It consisted of small microscopic foci composed of 5 to 20 adipocytes. In 13 cases, the fat was intimately associated with benign glands. In another 2 cases, it was associated with small nerves, and in 2 cases was random with no specific localization. Intraprostatic adipose tissue was located in the peripheral zone in 15 cases and in the central zone in 2. Intraprostatic adipose tissue, although uncommon, does exist. Therefore, caution must be exercised in diagnosing extraprostatic extension based only upon identification of fat invasion, especially in a needle biopsy. The small size of foci of adipose tissue and its admixture with benign glands are useful morphologic clues in distinguishing it from extraprostatic fat. [Copyright &y& Elsevier]

Summary: T3 renal cell carcinoma (RCC) is a heterogeneous group of tumors that are substaged based on perirenal or sinus fat invasion, adrenal invasion, and renal vein invasion. To evaluate whether the extent of fat invasion (minimal versus extensive) and direct adrenal gland invasion, renal vein invasion with or without concurrent fat invasion has a similar prognosis, we retrospectively reviewed 198 T3N0/NxM0 RCCs in a single academic tertiary hospital. Fat invasion was subdivided as minimal (≤5 mm into the fat) or extensive (>5 mm) invasion. Direct adrenal invasion was defined as contiguous involvement of ipsilateral adrenal gland. Among the 198 T3 RCCs, minimal and extensive fat invasions were identified in 57 and 61 cases, respectively; renal vein invasion and direct adrenal invasion were seen in 66 and 14 cases. The patients'' average age was 62.9 years, and 145 patients were male and 53 were females. The 2-year and 5-year survival rates were 85% and 56% for minimal fat invasion, 76% and 70% for extensive fat invasion, and 55% and 32% for renal vein invasion, respectively. There was no difference of survival in patients with T3b (renal vein invasion) RCC stratified by presence or absence of concurrent fat invasion. The 2-year and 5-year survival rates for adrenal invasion were 31% and 21%, respectively, which was significantly worse than that of fat or renal vein invasion. Multivariate analysis showed that nuclear grade, sarcomatoid differentiation, and subgrouping of pT3 RCC (fat invasion, renal vein invasion, and adrenal invasion) remained independent predictors of patient''s overall survival. In conclusion, our study shows that T3 RCCs with minimal or extensive perinephric fat invasion has a similar prognosis and is significantly more favorable than that of renal vein invasion regardless of presence or absence of concurrent fat invasion. In contrast, tumors with adrenal gland invasion carry a far worse prognosis than perinephric fat or renal vein invasion and thus supporting a separate stage category. [Copyright &y& Elsevier]

Abstract: Myxoid adrenal cortical neoplasms are rare, and to our knowledge, only about 23 cases have been reported in the literature, including 13 carcinomas and 10 adenomas. We recently experienced 4 cases of myxoid adrenal cortical neoplasms (3 benign and 1 borderline malignancy) and studied the clinical, histopathological, and immunohistochemical features of these neoplasms. There were 2 male and 2 female patients (age range, 37-61 years, mean, 48 years). All but 1 patient had hormone-related symptoms. The tumors weighed from 24.1 to 94 g (size, 4.1-9.8 cm). They were variably encapsulated with areas of hemorrhage. Histologically, the tumor cells were arranged in delicate arborizing cords or trabecula with myxoid areas varying from 30% to 70%. Three tumors were benign and 1 was of borderline morphology with mitoses of 3/10 high-power fields and mild to moderate nuclear pleomorphism. Two cases contained areas of myelolipomatous component. The tumor cells were positive for vimentin, synaptophysin, and inhibin but negative for cytokeratin. All patients are alive with no recurrence of their tumors or evidence of metastasis (follow-up of 14-20 months). Myxoid changes in adrenal cortical neoplasms are rare but can be seen in both an adenoma and a tumor of uncertain malignant potential. Because of prominent myxoid changes, other myxoid tumors occurring in the retroperitoneum should be excluded. The usual clinical and histological features can be applied to classify the lesions as benign, borderline tumor, or malignant. In our series, there was no case with frank malignant tumor. [Copyright &y& Elsevier]

Abstract: Eosinophilic angiocentric fibrosis (EAF) is an uncommon inflammatory fibrosing lesion of the upper respiratory tract and orbit that occurs mainly in young to middle-aged women. The etiology of EAF is unknown. To our knowledge, approximately 28 cases have been previously reported in the English literature. We report here 3 additional cases of EAF of the sinonasal tract; 2 in women aged 19 and 31 years, and 1 in a man aged 49 years. The 19-year-old woman is the youngest patient with EAF ever described. The patients presented with a nasal cavity mass, face pain, or nasal obstructive symptoms of long duration. [Copyright &y& Elsevier]

Abstract: To investigate the incidence of amyloidosis of seminal vesicles and ejaculatory system including ejaculatory ducts and vasa deferentia, we reviewed the whole mount sections of 447 radical prostatectomy specimens removed for prostatic cancer, including 273 cases from the United States and 174 cases from Korea. Of these, 21 cases (4.7%) showed amyloidosis in seminal vesicles, vasa deferentia, and in ejaculatory ducts. Ten of these (3.7%) cases were from the United States and 11 cases (6.3%) from Korea. The patients'' age ranged from 51 to 79 years (mean, 66.1 years). Amyloid deposition was found in 5 patients in the sixth decade (3.4%), 9 patients in the seventh decade (4.7%), and 7 patients in the eighth decade (9.3%). At the seventh decade of life, the Korean patients showed a higher incidence (8.3%) than American patients (2.5%), but other age groups showed no difference. All cases showed bilateral involvement of the seminal vesicles and ejaculatory systems. The deposits of amyloid tended to be nodular and affected the subepithelial region of seminal vesicles, vasa deferentia, and ejaculatory ducts. There was no amyloid deposit around blood vessels or in the prostatic parenchyma. Localized amyloidosis of the ejaculatory system involves not only the seminal vesicles but also the vasa deferentia and the ejaculatory ducts. The vessels or prostatic stroma are not part of this process. Amyloidosis develops subepithelially spreading to include the wall of these organs and appears to be related to advanced age. The incidence of amyloidosis of the ejaculatory system in Korean patients was higher than in US patients. [Copyright &y& Elsevier]

Summary: Primary cardiac sarcomas are exceptionally rare. We present a 10-year, single-institution experience with 24 primary adult cardiac sarcomas. These cases were retrieved from the Department of Pathology data file of the Methodist Hospital at Houston, TX. Clinical presentation and pathologic features were analyzed. Histologic classification was followed according to the criteria set by the World Health Organization, and grading according to the system proposed by the Federation Nationale des Centres de Lutte Contrele Cancer. There were 14 men and 10 women (male/female, 1.4:1) with a mean age of 42.2 years (range 20-68 years). The tumors involved the right atrium in 14 cases, left atrium in 6 cases, right ventricle in 2 cases, and left ventricle in 2 cases. The tumor size ranged from 2.0 to 17.0 cm (mean 7.2 cm), and, histologically, there were 10 angiosarcomas, 9 unclassified sarcomas, 3 synovial sarcomas, and 2 leiomyosarcomas. All 10 angiosarcomas originated from the right atrium, whereas 5 of the unclassified sarcomas were from the left atrium. Although cases were limited, no predilection site was found for the other histologic types. All tumors were graded as 2 (5 cases) or 3 (19 cases) in differentiation. The prognosis was poor with a median survival time of 25 months after diagnosis. The grade was not statistically significant on survival (P = .14). In conclusion, angiosarcoma and unclassified sarcomas are the most common sarcomas of the heart accounting for 76%, but rare tumors such as synovial sarcoma and leiomyosarcoma may also occur in this organ. The survival of cardiac sarcomas is poor. [Copyright &y& Elsevier]

Abstract: Although calcifications in the prostate are a common manifestation, the relationship between calcifications and prostate cancer is not clearly documented as in breast cancer. In addition, anatomical distribution of calcifications by zones of the prostate and ejaculatory system has not been systematically studied. To study the frequency and patterns of calcifications within the prostate and ejaculatory system, we reviewed the whole mount sections of 298 consecutive prostatectomy or cystoprostatectomy specimens. Calcifications were evaluated in the prostate (central, peripheral and transition zones, and verumontanum), ejaculatory ducts, and seminal vesicles. We graded the degree of calcifications as mild, moderate, or severe. Calcifications in the prostate and ejaculatory system were common, and their frequency in our series is as follows: 88.6% (264/298) of prostates, 58.1% (173/298) of seminal vesicles, and 17.1% (51/298) of ejaculatory ducts. The prostatic calcifications occurred mostly in benign glands and/or stroma of all zones and the verumontanum. Calcifications were more common in the transition zone than other zones. There were 4 cases of prostatic calcifications in the areas of prostatic adenocarcinoma: 3 cases with calcifications in the tumor glands and 1 case with calcifications in tumor stroma but not in the accompanying tumor glands. In conclusion, calcifications are a very common finding in prostatectomy specimens and seem mostly to be associated with benign prostatic hyperplasia. However, calcifications can occur in direct association with prostatic adenocarcinoma, although the incidence of this association is not as high as in breast carcinoma. Also, ejaculatory system calcifications are not an infrequent finding. [Copyright &y& Elsevier]

Summary: We present 2 cases of mixed epithelial and stromal tumor of the kidney with sarcomatous transformation. One patient was a 53-year-old woman who presented with macroscopic hematuria. The resected tumor involved the right renal parenchyma, measuring 13.0 × 8.0 × 4.0 cm, and extended to perirenal adipose tissue. The second patient was a 56-year-old woman who presented with right flank colic pain. The tumor measured 6.0 × 5.5 × 4.0 cm, with an intact capsule at the upper pole. Both tumors showed a well-circumscribed, multilocular, cystic, and focally solid mass. Sections of both tumors revealed benign and malignant components. The benign component consisted of multilocular cysts and fibrous stroma with a focally ovarian stromalike component. The malignant component in both cases was predominantly composed of undifferentiated cellular spindle cell sarcoma with frequent mitoses. One case showed additional heterologous malignant elements, including rhabdomyosarcomatous, chondrosarcomatous, and focal carcinomatous components. We report 2 additional cases of sarcomatous transformation in mixed epithelial and stromal tumor of the kidney. [Copyright &y& Elsevier]

Abstract: The incidence of ectopic Leydig cells (LCs) has been reported to be 40% to 90%. Several theories have been proposed to account for ectopic LCs, including in situ differentiation, migration from testicular interstitium, and trapping of peritubular LCs in the tunica propria of the seminiferous tubule during its thickening. To document the nature of ectopic LCs and to compare them with interstitial LCs, Sertoli cells, epididymal cells, mesothelial cells, and epithelial cells in rete testis, immunostainings with calretinin, CD10, inhibin, CK7, and CK20 were performed in 40 cases of orchiectomy specimens using tissue microarray sections. In addition, the frequency of ectopic LCs was evaluated. Of the 40 orchiectomy specimens, 14 cases demonstrated ectopic LCs with an incidence of 35%. Inhibin was positive in more than half of ectopic LCs (9/14, 64.3%) and in almost all interstitial LCs. Calretinin was positive in most of ectopic LCs (12/14, 85.7%) as well as in most of normally located interstitial LCs and mesothelial cells and some of rete testis epithelial cells. The ectopic and interstitial LCs as well as Sertoli cells were negative for CK7, CK20, and CD10. CK7 was positive in all epididymal cells and in most mesothelial cells and epithelial cells in rete testis. CD10 was positive in some of epididymal cells and epithelial cells in rete testis. CK20 was negative in all cells in the testis and epididymis. Ectopic LCs showed similar staining patterns to interstitial LCs with positive immunoreactivity for calretinin and inhibin. In this study, the frequency of ectopic LCs was 35%. The lower incidence in this study was most likely because of the limited sampling. Immunohistochemically, ectopic LCs showed identical immunohistochemical patterns with those of interstitial LCs. Calretinin appeared to be more sensitive but less specific than inhibin for LCs. Because calretinin is frequently positive in cells other than ectopic or interstitial LCs, a precaution is required to differentiate LCs from mesothelial cells and rete testis epithelial cells. Based on our study, we could not add anything else of what is known in regard to the histogenesis of ectopic LCs. The current theories including in situ differentiation, migration from testicular interstitium, and trapping of peritubular LCs in the tunica propria of the seminiferous tubules during its thickening seem to be valid histogenetic theories. [Copyright &y& Elsevier]

Abstract: Although the presence of the muscularis mucosae (MM) in the urinary bladder is well known, hyperplastic MM (HMM) has not been well established in the English literature. In this study, we analyzed the presence and distribution of the MM and HMM in 102 consecutive cystectomy specimens and 17 bladders obtained at autopsy. Hyperplastic MM, defined as MM composed of more than 3 layers of muscle fibers appearing either as fibers parallel to the surface mucosa or rounded bundles, was found in all of the cystectomy specimens. Hyperplasia was subclassified as mild (4 to 6 layers of smooth muscle) and prominent (more than 6 layers). Hyperplastic MM was found in the dome, trigone, and anterior, posterior, left lateral, and right lateral walls. The incidence was relatively similar among these locations. The 3 patterns of HMM (continuous, interrupted, scattered) were similar to those of MM. Hyperplastic MM appeared most frequently in the scattered pattern and was always adjacent to large-caliber blood vessels. The hyperplasia is usually mild and is occasionally prominent. The presence of HMM did not appear to be related to the patient''s age and occurs slightly more often in bladders from cystectomy for neoplasms than bladders obtained from autopsy. Although the MM has been used along with the muscularis propria as a histological landmark for staging of bladder cancer, little is known about HMM. Pathologists should be aware of its occurrence and morphologic patterns to accurately assess the depth of invasion of bladder tumors and avoid misinterpreting HMM as the muscularis propria. [Copyright &y& Elsevier]

Summary: Pseudosarcomatous fibromyxoid tumor (PFT), postoperative spindle cell nodule (PSN), sarcoma, and sarcomatoid carcinoma of the bladder are frequently difficult to distinguish histopathologically with significant differences in disease-related outcomes. A retrospective review of our pathology registry over the last 25 years identified 7 PFT, 10 PSN, 18 primary bladder sarcomas, and 17 sarcomatoid carcinomas. Most patients with PFT, PSN, sarcoma, and sarcomatoid carcinoma were diagnosed between the ages of 50 to 60 years with PFT and PSN most commonly detected in women. A previous history of urological instrumentation and bladder cancer was present in all patients with PSN but none of the patients with PFT. Pseudosarcomatous fibromyxoid tumors were characterized by a tissue culture-like proliferation of myofibroblastic cells with focal atypia and overall cytoarchitectural features mimicking nodular fasciitis. Sarcomas and sarcomatoid carcinomas exhibited cellular atypia, mitotic activity with atypical mitosis, and the presence of necrosis. Transurethral resection was sufficient to control all PFT and PSN with no evidence of distant metastatic spread. In contrast, local recurrences and distant metastases frequently occurred in patients with primary sarcoma and sarcomatoid carcinoma despite aggressive surgical management, which was often combined with neoadjuvant chemotherapy (50% and 65% disease-specific mortality, respectively). Pseudosarcomatous fibromyxoid tumor and PSN have unique clinical and pathologic features that allow their distinction from primary bladder sarcoma and sarcomatoid carcinoma. [Copyright &y& Elsevier]

Abstract: We report on a rare case of synchronous angiomyolipoma (AML) and 2 oncocytomas (OCs) in the same kidney of a 73-year-old woman. During the course of a follow-up for a long-standing staghorn calculus in the left kidney, a 4-cm right suprarenal mass and a small solid nodule adjacent to a cyst in the midportion of the right kidney were radiographically discovered. A radical nephrectomy was performed. The suprarenal tumor was found to be an AML, and the small nodule was an OC (1.5 cm). An additional OC (0.8 cm) elsewhere in the right kidney was also identified. The coexistence of AML and other renal tumors is uncommon. Among reported cases, conventional renal cell carcinoma, possibly reflecting its higher incidence, has been the most common concurrent tumor with AML. Although only rare cases of simultaneous AML and OC have been reported in the literature, it appears that the proportion of OC among concurrent renal tumors with AML is higher than expected based on its frequency among all surgically resected renal neoplasms. Whether there is a pathogenetic association between AML and OC needs to be further investigated. [Copyright &y& Elsevier]

Abstract: Mucinous tubular and spindle cell carcinoma is a rare and newly described type of renal cell carcinoma (RCC) with a relatively indolent behavior. However, its histogenetic origin or line of differentiation remains unclear. Twelve cases of mucinous tubular and spindle cell carcinoma were identified and retrieved from the files of 3 institutions. Detailed morphological features, as well as their immunohistochemical profile established with markers of proximal renal tubules (RCC marker antigen, CD15, and α-methylacyl-CoA racemase) and of distal renal tubules (kidney-specific cadherin and cytokeratin 7), were studied. The age range of the patients was 35 to 73 years with a median of 56 years. The male to female ratio was 1:3. All of the patients were alive with follow-up between 4 and 38 months. All the tumors were confined to the kidney with a mean tumor size of 6.9 cm (range, 1.8-17 cm). The tumors were composed of variable proportions of tubular and spindle cell areas with focal to prominent mucinous or myxoid stroma. Foamy macrophages were seen in 10 cases and were prominent in 4 cases. A focal compressed tubulopapillary growth pattern was seen in 10 cases. The tumor cells were uniformly cuboidal with ovoid to round nuclei and inconspicuous nucleoli (Furhman nuclear grade 3 in 6 cases). Focal necrosis was seen in 3 cases. Immunostains showed that tumors were positive for RCC marker antigen (11/12), α-methylacyl-CoA racemase (11/12), CD15 (8/12), CD10 (2/12), kidney-specific cadherin (1/12), and cytokeratin 7 (11/12). Its morphological features as well as a strong preferential expression of proximal tubule markers suggest that this tumor is a type of RCC with proximal tubular differentiation, which appears closely related to or represents a morphological variant of papillary RCC. [Copyright &y& Elsevier]

: ObjectivesTo evaluate whether the presence of, or the number of cores containing, high-grade prostatic intraepithelial neoplasia (PIN) found in men who underwent initial extended multisite biopsy could predict which men would have prostate cancer on subsequent repeat biopsies.: MethodsBetween June 1997 and January 2003, 1086 men underwent initial prostate biopsy for early detection of prostate cancer using an extended multisite biopsy scheme. Of these, 175 men without cancer underwent at least one repeat biopsy (range one to three; median interval between biopsies, 3 months). Among these 175 patients, 47 had high-grade PIN on initial biopsy.: ResultsThe initial extended biopsy identified cancer in 33.8% (367 of 1086) and high-grade PIN in 20.8% (226 of 1086). The incidence of high-grade PIN only in patients found to have cancer on initial biopsy was 29.7% (109 of 367). The presence of high-grade PIN was associated with concurrent prostate cancer at the initial biopsy (P <0.0001). Overall, repeat biopsy identified cancer in 18.3% of the 175 men. Of the 47 men with high-grade PIN, 5 (10.6%) were found to have cancer on repeat biopsy. The number of biopsy specimens positive for high-grade PIN on initial biopsy was not associated with the likelihood of prostate cancer on repeat biopsy. Multivariate logistic regression analysis showed that neither the presence of high-grade PIN nor the number of cores containing high-grade PIN on initial biopsy were predictors for prostate cancer on repeat biopsy.: ConclusionsThe number of cores positive for high-grade PIN was not predictive for cancer on repeat biopsy. [Copyright &y& Elsevier]

Abstract: Hernia sacs are generally regarded as routine specimens in the daily practice of surgical pathology. However, unexpected findings including carcinoma can occasionally arise in these seemingly benign specimens. To ascertain the prevalence of metastatic carcinoma found within hernia sacs and to determine the importance of routine histologic examination of hernia sacs, we conducted a retrospective study of all hernia sacs with a diagnosis of metastatic carcinoma reported in our hospital system between January 2006 and December 2012. Of 3117 total herniorrhaphy specimens between 2006 and 2012, 11 (0.35%) were found to have metastatic carcinoma. Interestingly, in 3 cases, the initial diagnosis of cancer was made during histologic examination of the hernia sac. Metastatic carcinoma in hernia sacs is a rare occurrence; however, it is encountered in routine practice. It is recommended that herniorrhaphy specimens not be discarded and, instead, be regarded as peritoneal biopsies for routine histologic examination. [Copyright &y& Elsevier]

Pleomorphic giant cell carcinoma (PGCC) of the prostate is a rare entity categorized as a variant of prostatic acinar adenocarcinoma in the 2016 World Health Organization (WHO) classification system. PGCC differs from conventional prostatic adenocarcinoma by having bizarre, markedly enlarged, and pleomorphic cells. It differs from high grade urothelial carcinoma by negativity for urothelial differentiation markers, and can be distinguished from sarcomatoid carcinoma by lack of spindle cells. Including two new cases described herein, there have been 51 cases of prostate PGCC reported in the English literature. Clinical features shared by cases of prostate PGCC include poor prognosis, occurrence in older patients, and frequent association with prior therapy. Pathologic features common to cases of prostate PGCC include admixture with a high-grade conventional prostate carcinoma component and absent or reduced expression of prostate differentiation markers. More recent studies have begun to elucidate the molecular characteristics of PGCC, detecting specific mutations and chromosomal translocations, and showing evidence of a high degree of molecular instability in these tumors. We report novel findings in two cases of PGCC including a PIK3CA p.His1047Arg mutation not previously described. One of our cases is the first to clearly demonstrate chronological loss of prostate markers during dedifferentiation from prior conventional prostate carcinoma to PGCC. Herein, we present our two new cases and comprehensively review the literature on all reported cases of PGCC with critical commentary on findings in cases of this rare tumor. • Prostatic PGCC is a rare entity recognized by the WHO 2016 classification. • PGCC must be distinguished from urothelial and sarcomatoid carcinoma. • PGCC often loses expression of typical prostate immunohistochemical markers. • Unique molecular features of PGCC are now being discovered. • We present novel findings in two cases and critically review the literature on PGCC. [ABSTRACT FROM AUTHOR]

Leiomyoma is a benign tumor of smooth muscle origin most common in areas of the body with abundant smooth muscle including the gynecologic, genitourinary, and gastrointestinal system. Leiomyoma outside of these locations is believed to arise from vascular smooth muscle and arrector pili muscles. Leiomyoma of an extremity is a rare diagnosis, especially when present in a digit of the hand due to the paucity of smooth muscle in this location. We report three cases of leiomyoma of a digit of the hand. • Leiomyoma is a rare but important differential diagnosis for benign masses found in the digit. • Leiomyoma can be found in association with the neurovascular bundle, tendon sheath, or confined to soft tissue. • Leiomyoma can show variable staining patterns for smooth muscle markers. [ABSTRACT FROM AUTHOR]

The 8th edition of the American Joint Committee on Cancer (AJCC) TNM staging system subdivides prostatic pT3 tumors into pT3a, which includes cases with extraprostatic extension (EPE) and pT3b, which is defined by the presence of seminal vesicle invasion (SVI) with or without EPE. Yet, it is not established whether combined SVI and EPE impart a worse prognosis compared to SVI alone. We studied a cohort of 69 prostatectomy patients with SVI with or without EPE. Patient age at the time of radical prostatectomy was documented and Gleason score and presence or absence of EPE and/or SVI were determined. Biochemical recurrence (BCR) was defined as a PSA rise >0.2 ng/mL. The frequency of BCR was 33.9% in cases with combined EPE and SVI versus 12.5% in cases with SVI alone (relative risk = 2.71). An additional cohort of 88 patients also showed a higher frequency of lymph node metastasis of 29% in patients with combined SVI and EPE at the time of radical prostatectomy versus a 10% frequency of lymph node metastasis in patients with SVI alone (relative risk = 2.9). Based on our data, we propose further subdividing pT3 prostate cancers into three groups: EPE alone (pT3a), SVI alone (pT3b), and combined EPE and SVI (pT3c). This classification system would more accurately identify patients with pT3 prostate cancer who are more likely to experience worse outcomes and provide clinicians with additional information to aid in follow-up and postoperative treatment decisions. • SVI and EPE affect prostate cancer prognosis. • Current AJCC staging does not distinguish combined SVI + EPE from SVI alone. • We found combined SVI + EPE showed higher rates of BCR than SVI alone. • Combined SVI + EPE also showed higher rates of lymph node metastasis • We propose "pT3c" to denote patients with combined SVI and EPE. [ABSTRACT FROM AUTHOR]

Abstract: Hibernomas are rare benign neoplasms composed of brown adipose tissue. Although they were first mentioned in the literature almost a century ago, relatively few reports have documented their range of pathological features. Multivacuolated fat cells with small, central nuclei are typical features of hibernomas. Based on the nature of the stroma and the appearance of the multivacuolated cells, 4 categories of hibernomas are described: typical, lipoma-like, myxoid, and spindle cell. The myxoid variant is very rare. In this report, we present and characterize the spectrum of morphological features of the myxoid variant of hibernoma by describing the case of a 45-year-old white man who was referred to our hospital for treatment of an ovoid mass in the posterior segment of the right deltoid region. Histopathologic examination of the resected specimen revealed a multilobulated, tan-yellow, variegated tumor with a rubbery consistency that was separated by thick fibrous septa with interspersed collections of foamy histiocytes. The mass was initially diagnosed as schwannoma with degenerative changes, but workup revealed a myxoid variant of hibernoma. This report highlights recent advances that may help confirm the diagnosis and explain the differential diagnosis of this rare tumor. This is one of only a few cases of a myxoid variant of hibernoma reported in the literature. [Copyright &y& Elsevier]

: ObjectivesTo evaluate whether two courses of chemotherapy after orchiectomy in patients with clinical Stage I nonseminomatous germ cell testicular tumor at high risk of relapse will spare patients additional chemotherapy or surgery.: MethodsHigh-risk patients had one or more of the following: preorchiectomy alpha-fetoprotein level of 80 ng/dL or greater, 80% embryonal cell carcinoma or greater, or vessel invasion in the primary tumor. Low-risk patients had none of these factors or had 50% teratoma or more without vessel invasion. High-risk patients were offered two 21-day courses of outpatient chemotherapy consisting of carboplatin, etoposide, and bleomycin. Low-risk patients and high-risk patients not receiving chemotherapy were observed.: ResultsOf 99 patients, we classified 76 as high risk and 23 as low risk of relapse. All but eight of the high-risk patients received chemotherapy. No patient who underwent chemotherapy developed relapse, although 1 patient with normal biomarkers and a late-appearing mass underwent retroperitoneal lymphadenectomy for mature teratoma. Two of the 23 low-risk patients had disease relapse; both successfully underwent chemotherapy. The nonhematologic toxicity was mild in patients receiving chemotherapy, and no patient required hospitalization. The median follow-up was 38 months (range 9 to 69).: ConclusionsTwo courses of postorchiectomy adjuvant chemotherapy were safe and well tolerated and markedly decreased the relapse rate in high-risk patients with clinical Stage I nonseminomatous germ cell testicular tumor without additional surgery or more protracted chemotherapy. This approach may avoid potential problems with compliance and diminish the cost of scrupulous follow-up. Our results support that surveillance for carefully selected patients at a low risk of relapse is appropriate. [Copyright &y& Elsevier]