Borderline Malignant Potential Research Articles

Inflammatory Myofibroblastic Tumor of the Esophagus and Stomach Successfully Treated With ALK Inhibitor in a Pediatric Patient: A Case Report and Concise Review of Literature.

An inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm of borderline malignant potential. Nearly half of all IMTs have rearrangement of anaplastic lymphoma kinase (ALK) locus on chromosome 2p23 which can be treated with targeted therapy. Herein, we describe an unusual presentation of IMT involving an anatomical region rarely implicated in this disease process. A 15-year-old male patient came to the ER with dysphagia and coffee ground emesis. On esophagogastroscopy, a nodular luminal obstructing 30 × 50 mm mass in the lower esophagus was found, which was continuous with a large, partially circumferential gastric mass extending from the mid-body to the proximal antrum. Biopsies from esophageal and gastric masses revealed submucosal lesions composed of cytologically bland spindle and epithelioid cells, intermingled with inflammatory infiltrate, for which several immunohistochemical (IHC) stains were performed. The molecular study demonstrated ATIC::ALK fusion. Based on morphological, IHC, and molecular study findings, the diagnosis of ALK-positive IMT was rendered. Because surgical excision was deemed infeasible, the patient was started on ALK-inhibiting therapy with crizotinib. The patient responded well with no evidence of residual or recurrent disease on follow-up imaging or surveillance esophagogastroduodenoscopy. Crizotinib was ultimately discontinued after 10 months of therapy, and the patient continues to undergo surveillance imaging for monitoring of disease burden.

Oncocytic adrenocortical neoplasm of borderline uncertain malignant potential diagnosed after robot-assisted adrenalectomy case report

BackgroundAdrenal incidentalomas are radiologically discovered tumors that represent a variety of pathologies, with the diagnosis clinched only on surgical pathology. These tumors may be clinically monitored, but triggers for surgery include size > 4 cm, concerning features on radiology, or hormonally functioning. Adrenal oncocytic neoplasms (AONs) are notably rare and typically nonfunctional tumors that are discovered as incidentalomas and exist on a spectrum of malignant potential.Case presentationWe discovered an exceptionally large (15 cm in the greatest dimension) incidentaloma in a 73-year-old man with left back pain and he was treated with robotic-assisted adrenalectomy. Surgical pathology was consistent with AON of borderline uncertain malignant potential; adjuvant mitotane and radiation were omitted based on shared decision-making.ConclusionLarge AONs are rare, usually benign tumors that can be safely treated with robotic-assisted adrenalectomy. Surgical pathology is the crux of diagnosis and post-operative management, as it informs both the initiation of adjuvant therapy and the stringency of post-operative surveillance.

Clinicopathological study of pseudomyogenic hemangioendothelioma

ObjectivesPseudomyogenic hemangioendothelioma (PHE) is a rare intermediate hemangioendothelioma. This article aims to study the clinicopathological features of PHE.MethodsWe collected the clinicopathological features of 10 new PHE, and examined their molecular pathological features by fluorescence in situ hybridization. In addition, we summarized and analyzed the pathological data of 189 reported cases.ResultsThe case group consisted of six men and four women aged 12–83 years (median: 41 years). Five instances occurred in the limbs, three in the head and neck, and two in the trunk. Tumor tissues were composed of spindle cells and round or polygonal epithelioid cells, which were either arranged in sheets or interwoven, along with areas of transitional morphology. Scattered or patchy stromal neutrophil infiltration was observed. Tumor cells had abundant cytoplasm, and some contained vacuoles. The nuclei had mild to moderate atypia, with visible nucleoli, and mitosis was rare. PHE tissues diffusely expressed CD31 and ERG, but not CD34, Desmin, SOX-10, HHV8 or S100, while some samples expressed CKpan, FLI-1 and EMA. INI-1 stain is retained. The proliferation index of Ki-67 is 10–35%. Seven samples were detected by fluorescence in situ hybridization, six of which had breakages in FosB proto-oncogene (AP-1 transcription factor subunit). Two patients experienced recurrence; however, no metastasis or death occurred.ConclusionsPHE is a rare soft tissue vascular tumor, which has biologically borderline malignant potential, local recurrence, little metastasis, and good overall survival and prognosis. Immunomarkers and molecular detection are valuable for diagnosis.

Superficial CD34 Positive Fibroblastic Tumour, A Mesenchymal Tumour with Borderline Malignant Potential: A New Entity

Superficial CD34 positive fibroblastic tumour (SCD34FT) is a distinct mesenchymal tumour of superficial soft tissues which has an intermediate malignant potential and is a recently introduced entity in the WHO classification. We present a case report of a 32-year-old male patient with SCD34FT. The tumour was located on right forearm and was essentially superficial in location. Wide local excision of the tumour was done. On histopathological examination, the tumour was composed of highly pleomorphic spindled as well as epithelioid cells with granular eosinophilic cytoplasm and numerous bizarre lobulated nuclear forms with few showing intranuclear inclusions. However, the mitotic count was very low. On immunohistochemistry (IHC), tumour cells were diffusely positive for vimentin and CD34 (characteristic of SCD34FT). During clinical follow-up, the patient is asymptomatic since the time of his surgery one year back. SCD34FT is a mesenchymal tumour of borderline malignant potential which is restricted to the superficial location.

Radiologically defined lipid-poor adrenal adenomas: histopathological characteristics

Adrenal lipid-poor adenomas (LPA) are defined by high unenhanced density (≥ 10 HU), and absolute and relative contrast medium washout > 60% and > 40%, respectively, at computerized tomography (CT). To date, no thorough histopathological characterization has been performed in those frequent lesions (one-third of adrenal adenomas). Our aim was to analyze the histopathological characteristics of adrenal LPA. Patients with LPA (n = 57) were selected among consecutive subjects referred for an adrenal incidentaloma or ACTH-independent Cushing syndrome. FluoroDeoxyGlucose-Positron Emission Tomography (FDG-PET) was performed in 37 patients. In patients treated by adrenalectomy (n = 17), Weiss score and Lin-Weiss-Bisceglia score (in tumors composed entirely or predominantly of oncocytes) were calculated. Radiological parameters did not differ among patients with ACTH-independent Cushing syndrome (n = 6) and those with adrenal incidentalomas associated with primary aldosteronism (n = 2), autonomous cortisol secretion (n = 14), or non-functioning (n = 35). Patients treated by adrenalectomy had larger tumors (28.9 ± 11.2 vs 17.3 ± 8.4mm, P < 0.001), higher CT unenhanced density (29.1 ± 11.0 vs 23.1 ± 9.0 HU, P = 0.043), and FDG-PET adrenal uptake (9.0 ± 6.4 vs 4.4 ± 2.3 SUV, P = 0.003) than non-operated ones. Oncocytic features > 75% of the tumor were detected in 12/17 cases (70.6%). Five of those showed borderline-malignant histopathological characteristics by Lin-Weiss-Bisceglia score. Among remaining non-oncocytic tumors, 1/5 had a Weiss score ≥ 3. Overall, 6/17 tumors (35.3%) had borderline-malignant potential. Radiological parameters were similar between patients with benign and borderline-malignant tumors. Adrenal LPA are a heterogeneous group of tumors, mostly composed of oncocytomas. Up to 1/3 of those tumors may have a borderline-malignant potential at histopathology.

Proliferações Melanocíticas de Potencial Maligno Incerto: Um Estudo Retrospetivo de 23 Doentes

Introduction: Diagnostic uncertainty is an inseparable component of medical practice. The dichotomous classification of melanocytic lesions as benign or malignant has been replaced in recent years by a more flexible approach in which diagnostic uncertainty is recognized and accepted.&#x0D; Methods: In order to characterize the population of melanocytic proliferations of uncertain malignant potential diagnosed at our center between 2007 and 2017, histopathological reports of melanocytic lesions classified, in this period, as either dysplastic or malignant were analyzed.&#x0D; Results: Twenty-three lesions (4.3%) with borderline histopathological features or uncertain malignant potential were identified. The mean patient age was 34.8 years. Sixteen lesions (69.6%) were observed by a second pathologist. The most frequently performed treatment was wide excision. A sentinel lymph node biopsy was performed on 2 patients, both being negative. The median follow-up time was 22 months, and there was no recurrence of any treated lesion.&#x0D; Discussion: The controversy of the subject extends from the nature and classification of these lesions to their treatment. In this work we perform a statistical review of the population of melanocytic proliferations of uncertain malignant potential diagnosed in our department.

CT and MRI Findings of Glomangiopericytoma in the Head and Neck: Case Series Study and Systematic Review.

Glomangiopericytoma is a rare sinonasal mesenchymal tumor of borderline or low malignant potential. We reviewed the CT and MR imaging findings of head and neck glomangiopericytoma via a retrospective case series study and systematic review. Our study revealed that glomangiopericytoma is a well-defined lobulated avidly enhancing soft-tissue mass with erosive bony remodeling that is most commonly found in the sinonasal cavity. Typically, it is hyperintense on T2-weighted images with vascular signal voids, has a high mean ADC value, and a wash-in and washout pattern on dynamic contrast-enhanced MR imaging. Although the CT findings are nonspecific, typical MR imaging findings, including those on the ADC map and dynamic contrast-enhanced MR imaging, may be helpful for differentiating glomangiopericytomas from other hypervascular tumors in the head and neck.

Rapidly growing juvenile granulosa cell tumor of the ovary arising in adult: a case report and review of the literature

BackgroundOvarian granulosa cell tumors (GCTs) are divided into adult GCT (AGCT) and juvenile GCT (JGCT). The AGCT is more common type, conversely, less than 5% of tumors are the JGCT and occur in mainly premenarchal girls and in women younger than 30 years. Although JGCT have different histologic features compared to AGCT, the two types have similar imaging features because they have similar gross appearance. Therefore, it is difficult to distinguish two types by radiologic findings. In addition, it has not been described about the growth rate of JGCTs in past literatures. The aims of this report were to describe a case of rapidly growing JGCT arising in adult with difficulty in diagnosing and to review the literatures.Case presentationA 38-year-old woman, presented with abdominal distension and frequent urination, was found to have a pelvic mass measuring approximately 12 cm on ultrasonography. On magnetic resonance imaging (MRI), right ovarian multiloculated cystic mass accompanied with hemorrhagic foci was demonstrated. Although the presumptive diagnosis of GCT was made based on MR findings, the intraoperative differential diagnoses included GCT, yolk sac tumor or malignant mucinous tumor due to cytologic atypia and lack of the typical findings for AGCT such as nuclear grooves and Call-Exner bodies. As a result, abdominal simple total hysterectomy, bilateral oophoro-salpingectomy, partial omentectomy and appendectomy were performed. Moreover, she had a history of laparoscopic uterine myomectomy about one year before, and during that surgery bilateral ovaries were found to be macrospically normal. Therefore, it was suspected the tumor became enlarged within the short period of time.ConclusionsEven though it is difficult to distinguish two types of GCT by imaging findings, in some cases without typical findings for AGCT pathologically, MRI could provide useful information in accurately diagnosing JGCT. Moreover, in this case, the tumor growth rate seemed to be rapid regardless of its borderline malignant potential. It may be related with nuclear atypia and high mitotic rate of the tumor.

Inflammatory myofibroblastic tumor of the lung: presentations of an uncommon finding

Inflammatory myofibroblastic tumor (IMT) is a rare occurrence most commonly affecting the pediatric population. It is considered a diagnostic challenge because of nonspecific clinical symptoms and radiologic presentations. IMT is a soft-tissue tumor that can occur throughout the body but is most commonly found in the lung. This article discusses the case of a 10-year-old girl who presented to our hospital with abnormal chest radiograph. Imaging revealed a heterogenous mass in the superior aspect of the left lower lobe. It was found incidentally when the patient presented to the emergency department. Surgical resection proved a diagnostic and therapeutic measure confirming diagnosis of IMT. The tumor has potential to cause illness either by its rare malignant potential or by mass effect. Because lesions are often asymptomatic with a predilection for pediatric patients, cases describing appropriate management are essential. IMT is a rare tumor of borderline malignant potential. IMT of the lung may invade hilar structures, the mediastinum, and the pleura. Lesions range in diameter from 1.5 to 14 cm. Etiology of the tumor is unclear but may include immune response to infection, trauma, or surgery. Affected patients may present with cough and shortness of breath but are often asymptomatic. Histology is unique but not specific. Immunohistochemistry can differentiate the tumor, by staining for overexpression of the anaplastic lymphoma kinase oncogene. In conclusion, surgical resection is the preferred diagnostic and therapeutic regimen for IMT. The majority of IMTs follow a benign course, but exceptions qualify as low-grade malignancies. As a result, it is important to identify, treat, and maintain vigilance. This case encompasses presentation, diagnosis, and treatment of pulmonary IMT and highlights best practices in addressing IMT for adolescent patients.

Multicystic mesothelioma has malignant potential: its grounds and mechanisms of peritoneal metastasis

From 2009 to 2016, 9 female patients with multicystic peritoneal mesothelioma (MCPM) underwent 11 cytoreductive surgeries (CRS). Mean age at diagnosis was 50.6 (range, 43-71). Mean peritoneal cancer index was 8.9 (range, 2~33). Most frequent peritoneal sector involved was pelvic peritoneum in 7 patients, and greater omentum was involved in 6 patients. Eight and 1 patients had complete CRS and incomplete CRS, respectively. Except 1 patient, 8 patients had multiple cysts on different peritoneal sectors, and diffuse involvement on peritoneal surface was found in 2 patients No patients had lymph node metastasis or extraperitoneal spread. However, 3 patients showed pushing invasion to small bowel mesentery, colon, umbilicus and stomach. Median- follow-up was 46 months (range 4-120). At the time of the present analysis, all patients were alive. One patient recurred in peritoneal cavity 47 month after complete cytoreduction. Peritoneal free-floating cysts (PFFC) were found in 8 of 9 (88.9%) patients. Sizes of PFFC ranged from 1 mm to 2 cm in diameter and the inner surfaces were covered with mesotheliallike cells. MIB-1 labeling rates of PFFC ranged from 0.1% to 2.0%. These results indicate that PFCC may attach on the orifice of subperitoneal lymphatic vessels, and may become metastatic. The present study strongly suggests that MCPM has a potentially malignant behavior. This category of disease is composed of 2 types: diffuse type and localized type with borderline malignant potential. PFFC have an important role in the formation of peritoneal metastasis.

Primary mucinous retroperitoneal tumour of borderline malignant potential – a case report

Primary mucinous retroperitoneal tumour of borderline malignant potential – a case report

Adrenocortical Oncocytoma With Borderline Malignant Potential Causing Subclinical Cushing Syndrome

ABSTRACT Objective: To report a rare case of large adrenocortical oncocytoma in a young patient that was successfully resected by a minimally invasive laparoscopic technique in a community hospital. Methods: Clinical case presentation and review of literature. Results: A 36-year-old male patient was found to have an 8-cm right adrenal mass on magnetic resonance imaging (MRI) of the lumbosacral region that was done to rule out lumbar disc disease. The patient was asymptomatic, but due to concerns of malignancy, further work-up was pursued. Biochemical endocrine studies demonstrated subclinical Cushing syndrome. The patient underwent laparoscopic right adrenalectomy. The final size of the mass was 11 cm, which was larger than the MRI reporting. The final pathology of the mass was consistent with adrenocortical oncocytoma with borderline malignant potential. Conclusion: Functioning adrenocortical oncocytoma with borderline malignant potential is a rare tumor. Only about 25 cases of functional adrenocortical ...

Necessity of appendectomy for mucinous borderline ovarian tumors. Systematic review.

The benefit of performing an appendectomy during the surgical treatment of a mucinous borderline ovarian tumor (mBOT) is still controversial, even though clinical guidelines recommend this procedure. Our aim was to systematically assess the available evidence on appendectomy in the case of diagnosis of mBOT. A comprehensive search of the literature was conducted using electronic databases using the search terms: (borderline OR low malignant potential) AND (appendectomy OR appendix). A total of 12 articles were retained after systematic review, including a total of 667 patients with borderline ovarian tumor. Appendectomy was performed in 232 of the patients presenting with a mBOT. Two (0.86%) appendiceal carcinomas were confirmed on pathological examination. Both appendices were grossly abnormal at the time of surgery. Our systematic review demonstrates that available evidence regarding the role of appendectomy in mBOT is fragmented and weak. Nevertheless, this review provides adequate evidence to highlight that appendiceal involvement in mBOTs appears to be extremely rare and microscopic appendiceal involvement is highly unlikely in apparently normal appendices. In spite of the diversity of the included studies, the lack of appendiceal involvement in all cases with apparently normal appendix is strikingly common. We can conclude that in the case of normal appearance of the appendix at the time of primary surgery, appendectomy is not mandatory. Furthermore, a patient with a normal appendiceal appearance during primary surgery with post-operative diagnosis of mBOT addition of a second look intervention seems unnecessary and even hazardous.

Giant Solid-Pseudopapillary Neoplasm of the Pancreas in a Child

Solid-pseudopapillary neoplasm of the pancreas (SPPNP) is a primary epithelial neoplasm of the exocrine pancreas with borderline malignant potential. The unusual features included the ‘giant’ size and the absence of metastases/ capsular or vascular invasion despite the large size and predominantly solid nature. At two years following complete excision, she is asymptomatic and the imaging shows no recurrence/metastases.

Recurrence of a functional adrenocortical oncocytoma of borderline malignant potential showing high FDG uptake on 18F-FDG PET/CT

Adrenocortical oncocytoma is a very rare tumor, which is not malignant and nonfunctioning in most cases. We report a case of a 53-year-old male with a 9.8 cm sized hyperfunctioning, well-encapsulated adrenal mass, which exhibited by high FDG uptake on a PET/CT scan. The patient had complained of symptoms of Cushing's syndrome for 4 months. Laparoscopic adrenalectomy was performed and the mass was pathologically confirmed as adrenocortical oncocytoma of uncertain malignant potential. Four years after surgery, the tumor recurred with distant metastases, which was proven by subsequent biopsy. 18F-FDG PET/CT also showed hypermetabolism in the recurred tumor and multiple metastatic lesions. Adrenocortical oncocytoma of borderline malignant potential with high FDG uptake may require long-term follow-up with clinical, hormonal, and imaging evaluations.

Recurrence of a carcinoid tumor of the ovary 13 years after the primary surgery: A case report

The current study presents the case of a patient with a recurrent carcinoid tumor of the ovary, 13-years after the primary surgery. The primary surgery consisted of a total abdominal hysterectomy and bilateral salpingo-oophorectomy for a left ovarian tumor at 54 years old. Pathologically, the tumor was diagnosed as a carcinoid tumor of the ovary. Following the primary treatment, the patient was admitted to a cardiologist due to carcinoid-induced heart failure. At 67 years old, the patient was referred to Kyoto University Hospital with a solitary mass 8 cm in diameter and located in the paraaortic area, which was detected by routine ultrasonography and subsequent computed tomography (CT) scans. Urinary 5-hydroxyindole acetate (5-HIAA), a serotonin degradation metabolite, was present at elevated levels. With a diagnosis of a recurrent carcinoid tumor, the patient underwent a tumor resection. The pathological diagnosis was that of lymph node metastasis of the trabecular carcinoid. Post-operatively, the 5-HIAA levels returned to normal. Carcinoid tumors occasionally recur following surgery due to borderline malignant potential. Due to the slow growing nature of these tumors, in specific cases, recurrence occurs following a long interval. Therefore, a relatively long follow-up period is required.

An Oncocytic Variant of Intraductal Papillary Neoplasm of the Bile Duct that Formed a Giant Hepatic Cyst

Intraductal papillary neoplasms of the bile duct (IPNB) is the collective term used to refer to papillary bile duct tumors, mucin producing bile duct tumors, and cystic bile duct tumors. Pathologically, these tumors may be considered a highly differentiated adenocarcinoma or a tumor of borderline malignant potential. IPNB is classified into one of four variants based on cell differentiation. The rarest, oncocytic, is characterized by oxyphilic granular cytoplasm and no mucous cell differentiation. The patient, a 59-year old man, was admitted with a complaint of abdominal fullness and a 30×25 cm cystic mass in the right hepatic lobe demonstrated on computed tomography (CT). The mass had no malignant features on CT or magnetic resonance imaging; however, a portion was FDG avid on 18F-fluorodeoxyglucose positron emission tomography scan (FDG-PET). A fenestration operation was performed for the presumed diagnosis of a hepatic cyst. Pathological examination of the cyst contents demonstrated some atypical cells suspicious for malignancy. After eight months of observation, abnormal FDG uptake was again observed at the residual cyst. A partial hepatectomy was performed to excise the cyst. Pathological examination demonstrated adenocarcinoma in situ derived from an oncocytic IPNB variant. Following the resection, the patient remained disease free for 40 months. This is an extremely rare case of an oncocytic variant of IPNB that was difficult to distinguish clinically from a solitary hepatic cyst.

Adenocarcinoma cells in effusion cytology as a diagnostic pitfall with potential impact on clinical management: A case report with brief review of immunomarkers

Distinguishing metastatic carcinoma cells from reactive mesothelial cells in effusion samples is often challenging based on morphology alone. Metastatic carcinoma cells in fluid samples may mimic reactive mesothelial cells due to overlapping cytological features. We report a case of a pleural effusion in a 51-year-old female patient with a medical history significant for bilateral ovarian tumors and peritoneal implants diagnosed as serous tumor of borderline malignant potential. The effusion was composed almost entirely of adenocarcinoma cells that morphologically mimicked reactive mesothelial cells. The diagnosis of metastatic adenocarcinoma was made after a wide immunostaining panel of antibodies. Recognizing metastatic adenocarcinoma cells in effusion samples can be challenging and an accurate diagnosis may have significant impact on clinical management as demonstrated by this case.

Oncocytic adrenocortical neoplasms—a clinicopathologic study of 13 new cases emphasizing the importance of their recognition

Oncocytic adrenocortical neoplasms (OANs) are a rare but important subtype of adrenal tumors with unique clinical and morphological features. We present 13 previously unpublished cases, of which 3 were classified as benign, 2 as having borderline malignant potential, and 8 as malignant according to the Lin-Weiss-Bisceglia criteria. Seven tumors (54%) showed evidence of endocrine activity. All were composed of more than 90% oncocytes confirmed immunohistochemically using the antimitochondrial antibody mES-13 and ultrastructurally in 4 cases. Small oncocytes were a frequent finding that challenges the conventional notion of oncocytes as necessarily having abundant cytoplasm. Most cases were immunoreactive for vimentin, synaptophysin, inhibin-α, melan A, and calretinin, the latter being a novel finding in this group of neoplasms. Cytokeratin positivity with AE1/AE3 and CAM5.2 was variable. The literature was comprehensively reviewed to identify all cases of OANs reported to date. Hormone production is not as uncommon as previously believed, occurring in 30%. The Lin-Weiss-Bisceglia criteria were retrospectively applied to all published cases with sufficient information and were shown to effectively separate tumors according to their future risk of recurrence and survival using Kaplan-Meier survival curves (log-rank test, P < .001 for both). The estimated overall median survival for malignant oncocytic neoplasms is 58 months (95% confidence interval = 27.5-88.5 months), providing the first preliminary evidence that the prognosis of malignant OANs is likely to be more favorable than conventional adrenocortical carcinomas, in which the reported median survival is between 14 and 32 months.

Borderline Phyllodes Tumor with an Incidental Invasive Tubular Carcinoma and Lobular CarcinomaIn SituComponent: A Case Report

Phyllodes tumors are an infrequent breast tumor presentation. A phyllodes tumor with a synchronous invasive ductal carcinoma is rarely described and has never been reported with lobular carcinoma in situ component. A 53-year-old female presented with a nine-year history of twice core biopsy proven fibroadenoma. After an increase in the tumor's growth velocity it was decided upon to undergo an excisional biopsy. Microscopic examination of the well-circumscribed pale-tan mass found focal areas of leaf like architecture with variable number of mitoses present, representing a phyllodes tumor of borderline malignant potential. Incidentally, at one edge of the mass was found a tubular carcinoma and lobular carcinoma in situ components. Thorough, routine follow-up of patients with biopsy proven benign breast masses is important to finding a masked malignant component.