Local Infiltrative Growth Research Articles

A case of trichoblastoma of the auricular cavity and review of the literature

Trichoblastoma（TB） is a rare germ cell skin adnexal tumor of the hair, and it is a rare follicular tumor of the skin that differentiates from the hair germ epithelium and is often regarded as a benign skin tumorHowever, it is poorly confined and has a local infiltrative growth pattern. tb occurs in the head and neck region, especially in the face, and presents clinically as a slow growing, well-defined and elevated nodule. TB is routinely treated surgically. Due to the lack of universally accepted treatment guidelines or protocols, the recurrence rate after surgery is high, which makes clinical cure more difficult. In this study, a 65-year-old female patient was found to have a swelling with recurrent rupture and pus flow from the right external auditory canal opening and the auricular cavity. After initial misdiagnosis as otitis externa, she was treated with conventional anti-infective therapy, but her symptoms did not resolve and gradually worsened before coming to our hospital. The condition presented in this case is relativelyrare,therepre,timely and accurate diagnosis and treatment are crucial for prognosis improvement of such diseases.

Acute pancreatitis as the initial manifestation of an ampullary neuroendocrine tumor.

Acute pancreatitis is a common condition in Gastroenterology. Among its possible etiologies are ampullary tumors, rare neoplasms whose growth can hinder pancreatic drainage. Although they are usually epithelial, adenomas and adenocarcinomas, less commonly other histological types have been reported, such as neuroendocrine tumors. They constitute a small percentage of both ampullary tumors and neuroendocrine tumors of the digestive tract, classified into three histological grades based on mitotic count and Ki-67. Although the diagnosis is usually incidental, its main form of presentation is clinical or analytical cholestasis, with acute pancreatitis being an exceptional initial presentation. Endoscopic resection is the treatment of choice for well-differentiated tumors without evidence of local infiltration of the duodenal wall or intraductal growth greater than 10mm, with endoscopic ultrasound playing a key role in this assessment. We present the case of a 45-year-old cholecystectomized woman who was admitted to our service with a condition compatible with acute pancreatitis, initially suspected to be of biliary origin. After several radiological and endoscopic studies, an enlargement of the duodenal papilla suggestive of ampulloma was detected. Histological examination demonstrated a well-differentiated neuroendocrine tumor of the duodenal papilla which, lacking evidence of local duodenal infiltration or intraductal growth, was successfully resected endoscopically.

Clinicopathological and molecular genetic features of lipofibromatosis-like neural tumor

Objective: To investigate the clinicopathological and molecular genetic features of lipofibromatosis-like neural tumor(LPF-NT). Methods: Three cases of LPF-NT in Children's Hospital of Fudan University from December 2018 to December 2019 were analyzed for their diagnosis and differential diagnosis. Results: One case was male and two cases were females, all of them aged under 1 years old. The clinical manifestation of LPF-NT was a local infiltrative growth mass in subcutaneous tissue. There were no specific radiologic features. Histologically, dense fascicles or compact sheets of spindle cells showed infiltration into subcutaneous adipose tissue. Spindle cells displayed a moderate degree of nuclear atypia with mitotic activity cells.Immunohistochemical study showed the tumor cells diffusely expressed S-100 protein, CD34, NTRKI, and pan-TRK. Fluorescence in situ hybridization detected rearrangement of NTRK1 gene in all 3 cases tested. Conclusions: LPF-NT is a newly named soft tissue tumor. Histological morphology, immunohistochemistry and molecular detection are helpful for the diagnosis and differential diagnosis of the disease.

Activated Signaling Pathways and Targeted Therapies in Desmoid-Type Fibromatosis: A Literature Review.

Desmoid-type fibromatosis (DTF) is a rare, soft tissue tumor of mesenchymal origin which is characterized by local infiltrative growth behavior. Besides “wait and see,” surgery and radiotherapy, several systemic treatments are available for symptomatic patients. Recently, targeted therapies are being explored in DTF. Unfortunately, effective treatment is still hampered by the limited knowledge of the molecular mechanisms that prompt DTF tumorigenesis. Many studies focus on Wnt/β-catenin signaling, since the vast majority of DTF tumors harbor a mutation in the CTNNB1 gene or the APC gene. The established role of the Wnt/β-catenin pathway in DTF forms an attractive therapeutic target, however, drugs targeting this pathway are still in an experimental stage and not yet available in the clinic. Only few studies address other signaling pathways which can drive uncontrolled growth in DTF such as: JAK/STAT, Notch, PI3 kinase/AKT, mTOR, Hedgehog, and the estrogen growth regulatory pathways. Evidence for involvement of these pathways in DTF tumorigenesis is limited and predominantly based on the expression levels of key pathway genes, or on observed clinical responses after targeted treatment. No clear driver role for these pathways in DTF has been identified, and a rationale for clinical studies is often lacking. In this review, we highlight common signaling pathways active in DTF and provide an up-to-date overview of their therapeutic potential.

Multiple Primary Recurrent Basaliomas (mPR-BCCs) of the Scalp with Cranial Bone Invasion.

We present a 68-year-old patient with multiple primary infiltrative BCCs in the scalp area, initially treated 14 years ago with superficial contact X-ray therapy, end dose 60 greys, followed by electrocautery (x2) several years later. He presented in the dermatologic policlinic for diagnosis and therapy of two additional, newly-formed pigmented lesions, and because of an uncomfortable, itchy, burning sensation in the area where lesions had been treated years before. Screening cranial computer-tomography (CT) examination revealed two deformities in the form of tumor-mediated osteolysis, affecting the diploe of the tabula externa on the left parietal and parasagittal areas. Complete excision with removal of periosteum and partial removal of the tabula externa was planned with neurosurgeons at a later stage. BCC is one of the most common malignant skin tumours of the head and neck region (about 90% of cases) and is characterised by a significant potential for local infiltration and destructive growth. Recurrent, invasive BCC of the scalp and calvarium is a difficult problem for which universally accepted treatment protocols had not been established. The primary treatment of aggressive BCCs is surgical, with a thorough examination of excision margins to ensure complete resection. Procedural-based options include standard excision, curettage, curettage with electrodessication, and Mohs micrographic surgery (MMS), with MMS being the gold standard for the definitive treatment of BCC. Improper removal or electrocautery (as in our case) of the several aggressive forms of BCC seems to be a particular problem, and not only for dermatologic surgeons. The risk of subsequent invasion and destruction of the cranium, underlying dura, and cranial nerves by basal cell carcinoma (BCC) is extremely low, with an estimated incidence of 0.03%, but is a potential complication over time. Computed tomography is the modality of choice for detecting tumour invasion into bone, which commonly appears as irregular demineralization or osteolysis.

Hepatic radiofrequency ablation: markedly reduced systemic effects by modulating periablational inflammation via cyclooxygenase-2 inhibition.

To determine whether celecoxib, a cyclooxygenase-2 (COX-2) inhibitor, can potentiate hepatic radiofrequency ablation (RFA)-induced local cellular infiltration and distant tumour growth. First, COX-2 expression was evaluated using immunohistochemistry in the local periablational rim 24h after hepatic RFA without/with intraperitoneal celecoxib in normal Fisher 344 rat liver. Next, local cellular infiltration of macrophages, stellate cells, and hepatocyte proliferation were quantified in C57BL6 mice 3-7d after RFA without/with celecoxib. c-Met, HGF, and VEGF levels after RFA were also measured. Finally, distant tumour growth and proliferation (Ki67 and CD34) were observed in subcutaneous R3230 tumours after hepatic RFA with/without celecoxib. Hepatic RFA-induced local activation of COX-2 was significantly suppressed using celecoxib. Celecoxib also reduced RFA-associated a) increased c-Met expression at 24h, b) HGF and VEGF levels at 72h, c) periablational macrophage and stellate cells at 3d, and d) hepatocyte proliferation at 7d. Similarly, celecoxib with RFA reduced distant tumour growth, tumour cell proliferation, and tumour microvascular density to sham levels, compared to increases observed with hepatic RFA alone. Increased activation of COX-2 after hepatic RFA contributes to periablational cellular infiltration and inflammation-mediated distant tumour growth, which can be successfully suppressed with a COX-2 inhibitor. • Thermal ablation of liver tissue can increase local inflammation and COX-2 expression. • Ablation-induced local inflammation can contribute to stimulation of distant tumour growth. • Local COX-2 inhibition with celecoxib can block ablation-induced distant tumour growth.

Basal Cell Carcinoma of the Skin to Tirana-Albania

Basal cell carcinoma (BCC) is the most frequent epithelial tumor of the skin, characterized by a local infiltrative growth in contrast to a very rarely metastatization. In rare cases this neoplastic process behaves aggressively with deep invasion and high recurrence risk after surgery. The pathogenesis and the factors that determine the extensive growth are not completely understood. The American Joint Committee on Cancer published on 1988 the morphologic characteristics necessary to define a BCC as giant. Only the 1% of all BCC achieves these parameters. Basal Cell Carcinoma is linked to a spontaneous mutation of PTCH gene situated in chromosome region 9q22.3. Environmental factors, as sun exposition, trauma or smoking, play an important role in the tumor development. The tumor on sets most frequently in the middle part of the face and scalp even that it can develop on all sun exposed skin. Aim of this study is to review the available literature on BBCs. Between April 2014 and March 2015, 50 patients with BCC were treated in the Department of Plastic and Reconstructive Surgery of Tirana. According to the AJCC classification, 40 tumors were T1 (80%), 5 tumors were T2 (10%) and 5 were T3 (5%). All patients received surgical excision of neoformation and immediate reconstruction. For small BCC (1-2cm), the surgical excisions require clinical margin of 3-5mm and the recurrence risk in this tumor is 4%-14%. Actually the gold standard is to perform a one-step or two-step surgery. In one step surgery the surgical defect is close directly and the reconstructive procedure is definitive. One step surgery, using Mohs Micrographic surgery, affords superior clinical outcomes, with a five-years tumor-free rate of over 99% for primary BCC and over 95% for recurrent BCC, compared to the rate of 93% and 80% respectively for other interventions. Collected data from our personal experience, according with the literature review, demonstrate that early diagnosis on association with the correct treatment is the only strategy to have a good prognosis of the disease. DOI: 10.5901/ajis.2015.v4n2s2p100

Complete Response to Exemestane in a Patient with a Desmoid Tumor

Desmoid tumors are rare mesenchymal neoplasms without metastatic potential. Despite the benign nature of this condition, some patients develop disease progression despite all locoregional options for care. Aggressive forms of desmoid tumors may induce morbidity that can lead to physical impairment and mortality that is occasionally observed as a result of local infiltrative growth and tissue invasion, in particular with abdominal disease. Few therapeutic options are available for patients with recurrent/unresectable desmoid tumors. Several studies have suggested the potential benefit of antiestrogens such as tamoxifen in this setting. Here we report the first description of the efficacy of an aromatase inhibitor in a patient with a desmoid tumor.

Solitary cylindroma with malignant transformation

A 59-year-old woman presented with a history of rapidly progressive recurrent tumor of 6.5 cm diameter of the scalp. Histopathological examination revealed a case of malignant cylindroma. There has been no recurrence or metastases and the patient is disease free at the end of 15 months postoperatively. Malignant transformation occurs less often in solitary form of cylindroma, but once transformed, tumors behave aggressively with extensive local infiltrative growth or metastases. The case is reported to document a rare case of malignant cylindroma arising in a patient with solitary cylindroma on the parieto-temporal region.

Limb Salvage Surgery in the Excision of a Massive Fibromatosis

Fibromatosis is a rare fibroproliferative disorder with a tendency for local infiltrative and destructive growth. Local recurrence is frequent, despite apparent complete resection after radical excision. We present a case of a 22-year-old woman with massive recurrent thoracic fibromatosis extending into the neck and impairing the function of her right upper limb. This required a multidisciplinary approach to surgery to salvage the limb. The case highlights the fact that while every attempt should be made to achieve negative histologic margins, local recurrence is not uncommon. Therefore, if fibromatosis occurs adjacent to or involves vital structures, these should not be sacrificed to achieve negative margins. Function and structure preserving procedures are important as the primary goal, if not even more important.

Tumor desmoide intraabdominal simulando una tumoración pancreática en una paciente con poliposis colónica familiar

Introduction Fibromatosis consists of a benign fibroblastic proliferation with local infiltrative growth. Two types are recognized: a superficial and a deep form, also known as desmoid tumor. The latter may occur sporadically or in association with familial adenomatous polyposis and Gardner's syndrome. Pancreatic presentation is exceptional and only eight cases have been described in the literature. Case report We report the case of a 29-year-old woman with a history of familial colonic polyposis and two pancreatic lesions. In the surgical specimen, two poorly defined pancreatic lesions were observed with infiltration of neighboring organs. Histologically, the lesions corresponded to mesenchymal proliferation with a fusocellular pattern without cytological atypica, which were diagnosed as desmoid tumors. Discussion The etiology of fibromatosis is unknown. In patients with familial colonic polyposis, the most common localization of desmoid tumor is intra-abdominal. Pancreatic presentation is unusual, requiring differential diagnosis with other pancreatic neoplasms.

Adénocarcinome polymorphe de bas grade : deux localisations palatine et labiale

Polymorphous low-grade adenocarcinoma (PLGA) is a variant of malignant tumor of minor salivary glands. We had for aim to discuss morphology, evolution and differential diagnosis of this rare tumor.The first case was a 65-year-old woman admitted for a two-month history of a right submaxillary swelling. The examination revealed a tumor of the right side of the palate. Biopsy was positive for a pleomorphic adenoma. The mass was enlarged and surgical resection was performed. The diagnosis was a partially resected PLGA. The patient also received adjuvant radiotherapy. There was a local recurrence 28 months after complete treatment. The second case was a 57-year-old woman who consulted for a 12-year history of lower lip swelling. The examination revealed a painless 2cm long located nodule in the mucosal side of the lower lip. An excisional biopsy was performed. The pathological examination concluded to a completely resected PLGA. The patient was free of disease at 54 months follow-up.The morphologic diversity and cytological uniformity of PLGA may make the differential diagnostic difficult especially with adenoid cystic carcinoma and pleomorphic adenoma. Its aggressiveness is proved by a local infiltrative growth pattern requiring a large surgical excision.

Therapieoptionen bei der aggressiven Fibromatose

Aggressive fibromatosis, e.g., desmoid tumour, is a rare neoplasm of the connective tissue with local infiltrative growth. Because of the high recurrence rates and destruction of the surrounding tissue, these tumours are classified as semi-malignant. However, desmoid tumors tend not to metastasise. Arising from deep musculoaponeurotic structures, a monoclonal proliferation of fibroblasts occurs. Radical surgical treatment with tumour excision accompanied by radiotherapy is the current standard therapy that can be supplemented by pharmacological treatment in a few cases. We report on 9 patients (5 males and 4 females) with surgical therapy for aggressive fibromatosis. The neoplasm was located on the extremities in 7 cases (4 x upper extremity, 3 x lower extremity), one tumour was situated in the chin and one in the rectus abdominis muscle. All patients were treated with radical tumour resection. Postoperatively, adjuvant radiotherapy was performed depending on the resection level of each patient. In 6 cases complete tumour resection (R0) was achieved. Following surgery, regular follow-ups with a physical examination and MR images were performed. In 3 cases a total tumour resection was impossible (R1 resection). The aggressive fibromatosis had infiltrated the pelvis in 2 cases implying a hemipelvectomy for R0 resection which was not practicable. Furthermore, diffuse locoregional infiltration of the chin region occurred in one case. In these cases, an adjuvant radiotherapy with 25 x 2 Gy was started postoperatively after the accomplished wound healing. Treatment with chemotherapeutic agents was not necessary. Aggressive fibromatosis is a semimalignant neoplasm of the connective tissue with an extremely high recurrence rate. Macroscopically, indistinct solid lesions are found with the destruction of muscles and other surrounding structures. Therapy of choice is the radical surgical resection. Radiotherapy is indicated for patients with non-resectable tumours. Pharmacological treatment should be considered for patients with unsuccessful local therapy.

Pulmonary inflammatory myofibroblastic tumor associated with nephrotic syndrome

Inflammatory myofibroblastic tumor (IMT) of the lung is a benign, non-metastasizing tumor with the possibility of local infiltration, recurrence or persistent local growth. This kind of tumor arises due to an unregulated growth of inflammatory cells. To our knowledge, IMT associated with nephrotic syndrome has not yet been recognized. Therefore, we present the case of a 14-year-old girl with lung IMT associated with secondary nephrotic syndrome (NS), which was cured after tumor removal.

Fibromatosis: Benign by Name but not Necessarily by Nature

Aggressive fibromatoses, also known as desmoid tumours, are rare fibrous tissue proliferations with a tendency for slow, local infiltrative growth. There is an association with Gardner's syndrome and familial adenomatous polyposis. Histologically they are fairly bland with no abnormal mitoses or necrosis. They do not metastasise, but can cause significant morbidity through their locally destructive effects. Magnetic resonance imaging is the method of choice for diagnosis, pre-treatment planning and post-treatment follow-up. Surgical excision with a wide margin is the treatment of choice. However, there is a tendency for local recurrence and repeated excision may result in a poor functional or cosmetic outcome. Radiotherapy is used to reduce local recurrence rates after excision and is also used to treat inoperable tumours. Long-lasting remissions can be obtained. Treatment is now planned using modern three-dimensional conformal techniques, similar to those used in soft tissue sarcoma management. There is no definite dose–response relationship, but doses of 50–60 Gy in 1.8–2 Gy fractions are recommended. Systemic therapy has been used for lesions not controlled by surgery or radiotherapy, or less commonly, as a primary treatment. Tamoxifen and non-steroidal anti-inflammatory agents are used most often as they are relatively non-toxic, but there is limited experience with cytotoxic chemotherapy and biological agents. There are no randomised trials to help guide the management of this locally aggressive ‘benign’ tumour and treatment decisions are best made by the local soft tissue sarcoma multidisciplinary team.

Desmoid tumor within lesser sac

Desmoid tumors or fibromatoses comprise a number of benign fibrous proliferative lesions that have local infiltrative growth and tendency to recur after incomplete excision. They never metastasize. The authors present a 31-year-old woman who, due to epigastric pain and palpable mass detected on presentation, underwent the excision of firm tumorous mass, 210 x 140 x 115 mm in diameter, from the lesser sac. Compressing the splenic vein, the tumor caused left-sided portal hypertension which subsided after the mass was removed. The recovery was uneventful. The histological examination verified typical desmoid tumor. Twelve years after surgery, the patient remained symptom-free with no signs of recurrence.

Inflammatory myofibroblastic tumour of the gallbladder

BackgroundInflammatory myofibroblastic tumour (IMT) is a benign, nonmetastasizing proliferation of myofibroblasts with a potential for local infiltration, recurrence and persistent local growth.Case reportWe report a case of a 51 year-old female, who had excision of a gallbladder tumour. Histopathology showed it to be IMT of the gallbladder.ConclusionThe approach to these tumours should be primarily surgical resection to obtain a definitive diagnosis and relieve symptoms. IMT has a potential for local infiltration, recurrence and persistent local growth.

Parathyroid hormone-related protein (PTHrP) expression in ameloblastoma.

Parathyroid hormone-related protein (PTHrP) production has been demonstrated in a variety of tumor subtypes. Local production of PTHrP by metastatic tumor cells in bone has been linked to bone destruction and tumor growth. Ameloblastoma (AB) is a relatively common odontogenic epithelial neoplasm that manifests local infiltrative intraosseous growth. AB recapitulates the developing enamel epithelium, in which PTHrP recently has been demonstrated. Yet PTHrP expression in a series of ABs has not been studied to date. The purpose of this investigation is to assess the expression of PTHrP in ameloblastoma. Formalin-fixed, paraffin-embedded tissue sections of ameloblastoma (n = 30; 24 conventional, 4 unicystic, and 2 arising in dentigerous cyst) were immunostained with anti-PTHrP antibody using a multistep streptavidin-peroxidase technique. Semiquantitative scoring of immunoreactivity was assessed as mild, moderate, and intense. All cases (100%) demonstrated positive immunoreactivity, with mild reaction in 3 conventional ABs, 1 unicystic and 1 AB arising in dentigerous cyst, and with moderate reaction in 12 conventional ABs, 3 unicystic and 1 AB arising in dentigerous cyst. Intense immunoreactivity was seen in 9 cases of conventional AB. This difference in immunostaining was not statistically significant (Sigma2 = 4.41, df = 4, P = .358). The results of this investigation suggest that PTHrP may play a significant role in local bone resorption, offering at least partial explanation for the tumor's infiltrative growth and destructive behavior. The uniformity of PTHrP expression by AB, as detailed in this study, may harbor significant therapeutic implications, particularly through PTHrP-blocking treatment modalities.

The diagnosis and treatment of periorbital sarcoid in the horse: 445 cases from 1974 to 1999

AbstractObjectiveDiagnosis and management of periorbital sarcoids in horses is a significant clinical challenge for the practicing veterinary surgeon and pathologist. The purpose of this study was to investigate the response of various types of sarcoids to different therapeutic methods.Animals studiedMedical records of 445 clinical patients.ProceduresThis paper retrospectively examines the clinical and histological features of periorbital sarcoids and the treatment of 445 cases. Treatment by surgical excision, cryosurgery, Bacillus—Calmette—Gaérin (BCG) immunomodulation, topical cytotoxic applications, and radiation are described.ResultsSix types of sarcoid can be found in the immediate periorbital region and each has some characteristics of other disorders of the skin with which they can be confused. The diagnosis of sarcoids is, however, relatively simple in most cases. The lesions are not necessarily restricted to the dermis and epidermis, but commonly invade into the subcutis and the deeper muscular structures around the eye. Treatment with radiation is expensive and difficult to manage but resulted in the best outcome with almost 100% resolution in 66 cases. By contrast, surgical excision has serious potential complications that arise primarily as a result of the infiltrative nature of periorbital sarcoids. The value of BCG immunomodulation therapy for fibroblastic and nodular lesions described by previous workers is confirmed in this paper, with a good overall response (69%), provided that the material was injected intralesionally. Perilesional injection did not appear to carry a significant benefit. However, treatment of verrucose or occult lesions by this method gave generally poor results. The results of intralesional injection of cisplatin emulsions suggest that this may be an effective method of treatment in cases where other modalities are not feasible. In common with previous reports, failure to resolve the lesions frequently resulted in regrowth of the tumor, and in most cases this recurrence appeared to be more aggressive, with extensive local infiltration and faster growth.ConclusionThe periorbital tissues are less tolerant of damage than those at many other sites and therefore certain treatment methods may be contraindicated. This suggests that it is important to select the best possible treatment at the first‐opportunity. It is a wise precaution to warn owners of horses with periorbital sarcoids of the dangers of leaving them and the particular risks associated with the currently available treatments.

The diagnosis and treatment of periorbital sarcoid in the horse: 445 cases from 1974 to 1999.

OBJECTIVE: Diagnosis and management of periorbital sarcoids in horses is a significant clinical challenge for the practicing veterinary surgeon and pathologist. The purpose of this study was to investigate the response of various types of sarcoids to different therapeutic methods. Animals studied Medical records of 445 clinical patients. Procedures This paper retrospectively examines the clinical and histological features of periorbital sarcoids and the treatment of 445 cases. Treatment by surgical excision, cryosurgery, Bacillus-Calmette-Gaérin (BCG) immunomodulation, topical cytotoxic applications, and radiation are described. RESULTS: Six types of sarcoid can be found in the immediate periorbital region and each has some characteristics of other disorders of the skin with which they can be confused. The diagnosis of sarcoids is, however, relatively simple in most cases. The lesions are not necessarily restricted to the dermis and epidermis, but commonly invade into the subcutis and the deeper muscular structures around the eye. Treatment with radiation is expensive and difficult to manage but resulted in the best outcome with almost 100% resolution in 66 cases. By contrast, surgical excision has serious potential complications that arise primarily as a result of the infiltrative nature of periorbital sarcoids. The value of BCG immunomodulation therapy for fibroblastic and nodular lesions described by previous workers is confirmed in this paper, with a good overall response (69%), provided that the material was injected intralesionally. Perilesional injection did not appear to carry a significant benefit. However, treatment of verrucose or occult lesions by this method gave generally poor results. The results of intralesional injection of cisplatin emulsions suggest that this may be an effective method of treatment in cases where other modalities are not feasible. In common with previous reports, failure to resolve the lesions frequently resulted in regrowth of the tumor, and in most cases this recurrence appeared to be more aggressive, with extensive local infiltration and faster growth. Conclusion The periorbital tissues are less tolerant of damage than those at many other sites and therefore certain treatment methods may be contraindicated. This suggests that it is important to select the best possible treatment at the first opportunity. It is a wise precaution to warn owners of horses with periorbital sarcoids of the dangers of leaving them and the particular risks associated with the currently available treatments.