Verteilungsmuster von Fuß- und Sprunggelenkstumoren

BackgroundUnclear bone lesions and soft tissue masses of the foot and ankle are not uncommon, but pseudotumorous lesions must be strictly differentiated from real neoplasia. Delayed and incorrect diagnoses are more common here than in other localizations of the musculoskeletal system, sometimes with serious consequences for affected patients.Awareness of true musculo-skeletal tumours is low due to the rarity of these diseases and neoplasia is often not considered.In the scarce literature on this topic there is no uniform definition of the term foot tumor, which makes it difficult to compare and correctly classify the different entities. The aim of this study is to show incidence and distribution patterns of foot and ankle tumors of a university tumor institute in order to improve the understanding of this rare and heterogeneous disease pattern, and to present a simple definition of the term foot tumor.MethodsIn a retrospective, monocentric study, data of patients treated between June 1997 and December 2015 for foot and ankle tumors at a musculoskeletal university tumor center were analyzed with respect to epidemiological data, entity and localization. This included all cases with foot and ankle tumors defined according to the WHO. Exclusion criteria were incomplete data on patient or entity and all pseudotumorous lesions.ResultsOut of a total of 7487 musculo-skeletal tumors, 413 cases (5.52%) of foot and ankle tumors were indentified in 409 patients (215 male and 198 female patients). The average age of the affected patients was 36 ± 18 years (min.3 years, max.92 years). There were 266 bone tumors (64%), among them 231 (87%) benign and 35 (13%) malignant tumors. Of 147 soft tissue tumors (36%) 104 (71%) were benign and 43 (29%) malignant. The most frequent benign osseous tumor lesions included juvenile / simple bone cysts, enchondromas and osteochondromas. By far the most frequent malignant bone tumor was chondrosarcoma. Common benign soft tissue tumors included tenosynovial giant cell tumor (including pigmented villo-nodular synovitis), superficial fibromatosis and schwannoma, while the most frequent malignant entities were synovial arcoma and myxofibrosarcoma. In terms of anatomical localization, the hindfoot was most frequently affected.ConclusionsKnowledge of incidence and the corresponding distribution patterns of foot and ankle tumors can help to correctly assess unclear bumps and lumps and to initiate the right steps in further diagnosis and treatment. Ignorance can lead to delayed diagnosis and inadequate treatment with serious consequences for the affected patient.     

Axial giant cell tumor - current standard of practice

Giant cell tumors of bone are relatively rare in the axial skeleton, accounting for approximately 6.7% of all cases. Due to their anatomical complexity, difficult access and proximity to vital neurovascular structures, management of these tumors poses a huge challenge on the treating surgeon. Several data series reported on axial GCTB involve short series of limited cases with varied methods used in their local control due to which, proper guidelines are unavailable for the management of such difficult cases. Though the present data support the use of denosumab for effective management of these lesions but there is varied consensus on dosage and duration of treatment. This review article summarizes the basic features and treatment modalities related to axial GCTB stressing on multidisciplinary approach to achieve optimum outcomes.     

Testicular neoplasms in the prepubertal male

Unlike adult neoplasms, prepubertal testicular tumors are frequently benign. Yolk sac tumors, which are always malignant, constitute the majority among the histological types of neoplasm reported in the literature. Teratomas are benign in the pediatric population and are the second-most commonly reported testicular tumors. The remaining tumors are exceedingly rare and almost always benign; however, juvenile granulosa cell tumors, undifferentiated stromal tumors, and Sertoli cell tumors may rarely possess metastatic potential. Therefore, testicular neoplasms are varied, rare and often benign. However, even when they are malignant, long-term survival is excellent with prompt diagnosis and treatment. Following an English-based Medline literature search, this article reviews a concise approach towards the management of these individual tumors based on an analysis of the literature in the English language that addresses testicular neoplasms in prepubertal males.     

Laparoscopic splenectomy for solitary splenic tumors

Background  Solitary splenic masses are a rare entity. There is a paucity of data in the literature on the evaluation and laparoscopic treatment for splenic masses. To further elucidate the evaluation and laparoscopic management of splenic masses we evaluated our own data. Materials and methods  Data was collected retrospectively for all patients who underwent laparoscopic splenectomy (LS) in our institution for the diagnosis of a solid mass. Patients’ charts were reviewed. Complementary data was completed when needed by telephone interviews. Results  28 patients underwent LS for solid splenic masses between 1997 and 2006. Mean age was 54.3 years and 68% were women. Patients’ symptoms included abdominal pain (46.5%), anemia (32%), weight loss (21%), and palpable abdominal mass (21%). Fifty-three percent were asymptomatic at diagnosis. Preoperative patients’ imaging included computed tomography (92.8%), abdominal ultrasound (71.4%), and positron emission tomography (PET, 32%). Seven patients (25%) had a history of lymphoproliferative disease. The mass size as measured by computed tomography (CT) scan ranged from 4 to 11 cm. Three patients (10.7%) had multiple splenic lesions. Mean operative time was 125 min. Mean estimated blood loss was 200 ml. Five patients (17.9%) had massive splenomegaly. Conversion rate was 14.3%. In three patients (10.7%) the spleen was removed with additional organs’ tissue (stomach and pancreas). Two patients (7.1%) were reoperated. There was no postoperative mortality. Mean hospital stay was 4.7 days. Four patients (14.3%) were readmitted due to complications. Pathology revealed eight patients (28%) with benign tumors and the rest (71.4%) with malignant lymphoma. Conclusions  Splenic solid tumor is a rare entity. Most of the cases were eventually diagnosed as malignant tumors. In our series, all malignant tumors were non-Hodgkin lymphoma. The most common benign lesion was inflammatory pseudotumor. This study has demonstrated the feasibility and safety of LS for diagnosis and treatment of both benign and malignant tumors of the spleen.     

Neuro-oncology of neurofibromatosis type 1

Opinion statement  Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with an incidence of about 1:2500 to 1:3000. It is caused by a germline inactivating mutation of the NF1 gene on chromosome 17. Patients with NF1 are at increased risk of developing a variety of tumors of the peripheral and central nervous system, including neurofibromas, plexiform neurofibromas, malignant peripheral nerve sheath tumors, and low-grade gliomas of the optic nerves and other cerebral structures. Rarely, they develop high-grade gliomas. Although they are rare, these hereditary tumor syndromes involving the nervous system must be recognized in patients and their families, as early diagnosis may alter management and ultimately improve outcome. Additional insight into the molecular mechanisms causing these syndromes and their relationship with the clinical features will allow the development and implementation of screening and prevention strategies for these diseases. Management of these lesions is difficult and requires specific skills and the collaborative work of neurosurgeons, radiation therapists, neurologists, and oncologists. Ideally, patients should be managed in comprehensive centers with specific expertise in the management of patients with NF1. This review describes current and developing therapies for managing the neuro-oncologic manifestations of NF1.     

Local recurrence after breast‐conserving therapy for phyllodes tumors: A 15‐year retrospective review

There are few data on the long‐term outcomes of patients with phyllodes tumors following breast‐conserving surgery with or without radiation therapy (RT). We reviewed 69 patients diagnosed from 2000 to 2015 with surgical specimens available for central pathology assessment for outcome in relation to histopathologic subtype, margin width, and utilization of RT. Median follow‐up was 63 months (interquartile range, 35‐131 months). Forty‐eight patients had benign, 13 borderline, and eight malignant phyllodes tumors, with local recurrence rates of 4%, 0%, and 38%, respectively (P ≤ .04 comparing malignant lesions to both benign and borderline lesions). None of the eight patients who received RT suffered a local recurrence. Two of the 26 (8%) patients with benign phyllodes tumors who did not receive RT with margins that were positive or <1 mm had local recurrence, compared to none of 18 patients with margins 1 mm or wider who did not receive RT. The one patient with a malignant phyllodes tumor who did not receive RT with margins that were positive or <1 mm did not locally recur, while both patients with margins 10 mm or wider who did not receive RT had local recurrence. One patient with a malignant phyllodes tumor developed distant recurrence following local recurrence. Phyllodes histologic type and margin width were both associated with the risk of local recurrence following breast‐conserving surgery without RT, though the number of events and patients was too small to show these trends were statistically significant.     

Wide local excision for anal GIST: A case report and review of literature

IntroductionGastrointestinal stromal tumors (GIST) are tumors of mesenchymal origin commonly detected in stomach and small bowel. GIST arising primarily from the anal canal is extremely rare. Due to the malignant potential, these tumors are treated with radical surgery like abdominoperineal resection. But with the advent of imatinib therapy and a better understanding of the tumor biology, some cases have been successfully treated with wide local excision.Presentation of caseWe describe a case of a 70-year-old lady presenting with a 2 cm mass in the anal canal. Endoanal ultrasound revealed a well-circumscribed solid nodule in the intersphincteric space. The patient was successfully treated by wide local excision and adjuvant therapy with imatinib mesylate.DiscussionOnly 14 confirmed cases of primary anal GIST have been reported in the literature. It appears as a well circumscribed hypoechoic mass arising from the intersphincteric space encroaching into the lumen on endorectal ultrasound. Lymphadenopathy is absent. Anal sphincters get involved as the lesion increases in size. Treatment is often planned based on the extent of the disease, the mitotic rate, patient’s general condition and willingness for a permanent colostomy.ConclusionSmall lesions (<2 cm) with low mitotic rate may be successfully managed by local excision. Radical surgery should be reserved for large, aggressive tumors.     

A case report of testicular diffuse large B-cell malignant lymphoma with cutaneous metastasis: A rare entity

Introduction and importancePrimary testicular lymphoma (PTL) is a variety of extra-nodal lymphoma taking origin from testis. It accounts 5% of all testicular tumors. Metastasis may occur in contralateral testis, bone, central nervous system and rarely in skin. Herein, we present the case of testicular diffuse large B-cell malignant lymphoma with cutaneous metastasis.Case presentationA 60-year-old male presented with swollen painless solid right testis, with homolateral inguinal nodes. Testicular tumors markers were within normal range. Right radical orchidectomy was performed. Histopathological examination concluded to the diagnosis of Diffuse Large B Cell Lymphoma. Four weeks later, the patient presented alteration of general condition and multiples cutaneous centimetric lesions located in the right inguinal region. Biopsy of this lesion confirmed the diagnosis of metastases from the testicular lymphoma. The patient deceased three days later, before starting further treatment.Clinical discussionPrimary testicular lymphoma is a rare variety of testicular tumors. The prognosis is poor. Metastasis may occur in different sites such as contralateral testis, central nervous system, and skin. The prognosis is usually poor in the rare case of cutaneous metastasis.ConclusionPrimary testicular tumor is an aggressive rare variety of testicular tumors with poor prognosis. Cutaneous metastasis is rarely reported. Cutaneous lesions should be explored and suspected to be malignant. Early treatment with rapid multidisciplinary management is the key for adequate approach.     

Testicular Microlithiasis Associated with Teratocarcinoma and Intratubular Germ Cell Neoplasia: A Case Report

Testicular microlithiasis is a rare condition that has characteristic sonographic and histologic features. It is often associated with premalignant changes and malignant neoplasms of the testes. We report a case of testicular microlithiasis associated with teratocarcinoma and intratubular germ cell neoplasia. Three previously reported cases documented development of malignant germ cell tumor during the clinical follow-up period of patients with testicular microlithiasis. We think that testicular microlithiasis is strongly associated with testicular neoplasms, and that coexistence of intratubular germ cell neoplasia and malignant germ cell tumors with testicular microlithiasis is common.     

Le médulloépithéliome du système nerveux central. À propos de trois cas

Central nervous system medulloepithelioma is a rare, highly malignant childhood tumor. It might be confused with medulloblastoma or other primitive neuroectodermal tumors, but it is quite particular by its clinical, radiological, and pathological features. The mean survival varies depending on whether or not a gross-total resection is possible. Adjuvant radiochemotherapy is often indicated. Only two reported cases in the literature survived beyond 4 years after treatment by gross total resection and radiotherapy without chemotherapy. We report three cases of supratentorial medulloepithelioma occurring in three children aged 11-17 years. Two patients underwent a gross-total resection followed by radiotherapy and survived more than 4 years after treatment. The third case had, however, recurred twice within the 1st postoperative month despite a complete resection each time and metastasis to the lung developed. Chemotherapy was then carried out after the third procedure and the patient died 7 months later.     

Clinical and pathological features associated with the testicular tumor of the adrenogenital syndrome

PURPOSE: Testicular tumor of the adrenogenital syndrome is a rare clinical entity found in young men with endocrine disorders. Histologically it resembles Leydig cell tumor. We 1) reviewed the clinical features of testicular tumor of the adrenogenital syndrome and 2) determined if special histopathological features of the tumor and synaptophysin reactivity could distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor. MATERIALS AND METHODS: We reviewed the medical and pathological records for all patients with testicular tumor of the adrenogenital syndrome seen at our institution from 1978 to 2004. These tumors were examined by histological and immunophenotypic methods for comparison to Leydig cell tumor. RESULTS: A total of 14 males with an endocrine disorder had pathological evidence of testicular tumor of the adrenogenital syndrome. These tumors were often bilateral (93% or 13 of 14 cases), associated with pain (92% or 12 of 13) and refractory to medical management with high dose exogenous steroids (93% or 13 of 14). Testicular tumor of the adrenogenital syndrome was managed by tumor enucleation in 7 patients (54%) and by radical orchiectomy in 6 (46%). All patients had resolution of pain at 3-month followup. Upon histological review features found to be more common to testicular tumor of the adrenogenital syndrome compared with Leydig cell tumor were nuclear pleiomorphism, low mitotic activity, extensive fibrosis, lymphoid aggregates, adipose metaplasia and prominent lipochrome pigment. Synaptophysin (ICN, Costa Mesa, California) reactivity was strong in testicular tumor of the adrenogenital syndrome but rarely observed in Leydig cell tumor. CONCLUSIONS: In our series medical treatment failed in patients with testicular tumor of the adrenogenital syndrome and conservative surgical therapy was possible in select individuals. We identified special histopathological and immunophenotypic features, including synaptophysin staining, which distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor.     

Diode laser photocoagulation of intraoral (and perioral) venous malformations: Cases series

Vascular anomalies are subdivided into vascular tumors (hemangiomas) and vascular malformations. They are frequently located in the head, neck, and oral cavity. They are common complaints reported in patients seeking treatment for aesthetic or functional issues.However, recent advances in the diagnosis and management of these lesions are improving treatment strategies. This review provides both basic and up-to-date knowledge on the most common vascular anomalies encountered by practitioners.Due to the wide variability of treatment options which often generates debate, this paper work aims to provide a comprehensive approach of these lesions based upon current concepts and practical clinical experience.Our article is about 4 patients who had consulted for one or several purplish, elevated, well limited and soft lesion. These lesions was not painful, but worrying for patients. Therapies for VAs continue to generate a dilemma for oral surgeons. Several treatment options were reported, including conventional surgery with or without adjunctive preoperative embolization, and drug therapies, such corticosteroids, intralesional injection of corticosteroids and intralesional injections of sclerosing agents. All of these therapeutic approaches carry a high risk of severe side effects such as scars, pain, and bleeding [7].Nowadays, advances in the use of lasers have allowed doctors an effective treatment with minimal side effects [9].All our cases described in this article were done by Pr Haitami, using the 980 nm Diode laser, and a complete healing was observed in about 8 months at the most. The laser is therefore a great help in the management of this type of lesion.     

Management of a periductal stromal tumor in a young woman: Our breast unit experience

Periductal stromal tumor (PDST) is a rare biphasic tumor of the breast that exhibits low‐grade malignancy and intermediate behavior. It is characterized by proliferation of atypical spindle cells surrounding benign mammary ducts and infiltrating adjacent adipose tissue. PDST is distinguished from phyllodes tumor by its lack of leaf‐like architecture; however, it is still unclear whether PDST is a separate entity or a certain spectrum of phyllodes tumor. Phyllodes tumors constitute a group of rare epithelial lesions of the breast which mainly develops at around 40‐50 years. The histologic characteristics to be considered are many and often heterogeneous in the same lesion which makes interpretation in needle biopsy material difficult. Most phyllodes tumors have a benign nature, with a high rate of postsurgical recurrence. In the malignant form, metastases are described by distant hematogenous route; its indolent behavior implies a tight surgical management with precise excision of the lesion even if there is not, however, a unanimous consent on the parameters of accuracy of the margins.     

Cisplatin, Vincristine, Methotrexate, Peplomycin, Etoposide (COMPE) Therapy for Disseminated Germ Cell Testicular Tumors

Background The advent of cisplatin rendered disseminated testicular germ cell tumor an often curable malignant disease. Patients with a heavy metastatic burden, however, remain poor risks; furthermore, many patients experience nausea or other adverse events. This paper reports a trial of a cisplatin-based (COMPE) combination chemotherapy regimen based on synchronization theory.
Methods Twenty patients with disseminated germ cell testicular tumors were treated with COMPE; any residual tumor mass was surgically resected.
Results Seventeen patients (85%) achieved complete remission by chemotherapy. The actuarial overall and cause-specific 3-year survival rates were 89% and 94%, respectively. In the subset of 16 "good-risk" patients, all remain alive after a median follow-up of 43 months. Complications were quite tolerable, with nephrotoxicity in particular being extremely mild.
Conclusion COMPE is an effective chemotherapy regimen in patients with disseminated germ cell testicular tumors. Complications arising from this therapy are mild.     

Transformación maligna secundaria de teratomas testiculares: serie de casos y revisión de la literatura

BackgroundTeratomas are a spectrum of neoplasms that can undergo malignant transformation. In the World Health Organization (WHO) classification of tumors, this entity was classified as «teratoma with somatic-type malignancy», was defined as a malignant neoplasm of non-germinal phenotype that originates in a teratoma.Materials and methodsWe present a serie of nine cases of testicular teratomas with secondary malignant transformation. From January 1995 to December 2011, we found a total of 306 cases of testicular tumors. Mixed germ cell tumors were the most frequently diagnosed malignancy with 45.7%.ResultsTeratoma with secondary malignant transformation, represented 2.9% of all germinal tumors. Five cases originated within a mixed germ cell tumor, two cases from mature teratomas, and two from immature teratomas. The predominante malignant somatic component were sarcomas; two cases of chondrosarcoma, one rhabdomyosarcoma, and one case showing foci of chondrosarcoma and rhabdomyosarcoma. The case of osteosarcoma is notable for its rarity. Two cases showed epithelial malignancy in the form of an adenocarcinoma, and finally, two cases were primitive neuroectodermal tumors. At the time of diagnosis, five patients had metastases.ConclusionThe transformation of germ cell tumors to somatic type malignancies is rare. The malignant component can originate from any of the three germ lines. These tumors are resistant to standard chemotherapy for a germ cell tumor and the clinical stage is the most important prognostic factor. At our institution, the malignant component that appeared most frequently was chondrosarcoma.     

Fibroinflammatory Biliary Stricture: A Rare Bile Duct Lesion Masquerading as Cholangiocarcinoma

Introduction  Fibroinflammatory biliary stricture (FIBS) is a rare benign tumor-like process of the extrahepatic bile duct that masquerades as cholangiocarcinoma. Methods  In order to distinguish this unusual entity from cancer, we performed a systematic analysis of 11 patients with FIBS. All patients presented with jaundice; six patients had coexisting autoimmune disease. Preoperative evaluation included computed tomography scan and endoscopic retrograde cholangiopancreatography with benign brush cytology. Surgical treatment included nine bile duct resections with five concurrent liver resections and two incisional biopsies. Light microscopy demonstrated fibrous lesions admixed with chronic inflammation. Results and discussion  Immunohistochemistry demonstrated smooth muscle actin expression in all lesions except one; five tumors exhibited IgG4 positive plasma cells. The lesions were negative for cytokeratin, ALK1, CD21, S100, Ki67, and p53. Six patients received postoperative immunosuppression. At 41 month median follow-up (range 15–58 months), there was no evidence of recurrent FIBS in ten patients, while one was lost to follow-up. Conclusion  FIBS is a rare myofibroblastic lesion with an immunohistochemical profile distinct from other epithelial and stromal neoplasms of the extrahepatic bile duct. A subset of these cases appear to represent IgG4-related sclerosing cholangitis. Because preoperative cytology is not diagnostic of FIBS, surgical resection remains the mainstay of diagnosis and treatment, while immunosuppression may reduce the risk of recurrence. Poster Presentation at Digestive Disease Week, May 2006, Los Angeles CA, USA.     

Demographics,management and treatment outcomes of benign and malignant retroperitoneal tumors in Japan

Objectives

To show the demographics, type of treatment and clinical outcomes of patients with retroperitoneal tumors in Japan.

Methods

We carried out a retrospective analysis of patients with retroperitoneal tumors treated between 2000 and 2012 at 12 university hospitals in Japan. Histology was re‐evaluated using the 2013 World Health Organization classification.

Results

A total of 167 patients were included in the analysis. The number of diagnosed patients increased over the 12‐year study period. Liposarcoma and schwannoma were the most common histological types among intermediate/malignant and benign tumors, respectively. The intermediate/malignant tumors were larger and were more frequently found in older people. Surgical resection was the primary treatment for 151 patients. The median survival duration for patients with malignant tumors was 91 months, and was significantly shorter than that for patients with benign and intermediate tumors (P < 0.01). R2 resection was associated with significantly shorter survival than R0/R1 resection for malignant tumors (P < 0.01), but not for intermediate. Grossly complete resection of the recurrent tumors improved survival.

Conclusion

The number of patients diagnosed with retroperitoneal tumors increased over time. R2 resection of primary tumors was found to be associated with poor prognosis in malignant tumors, but not in intermediate tumors. Complete surgical resection of recurrent tumors was associated with a better oncological outcome.     

Non-germ cell tumors of testis

Testicular tumors of non-germ cell origin represent from 5 percent to 10 percent of all testicular neoplasms. Included in this group are sex cord/gonadal stromal tumors, most originating from Leydig or Sertoli cells, mixed tumors, and tumors of mesenchymal or hematopoietic origin. In addition, various miscellaneous lesions, tumor-like conditions, and secondary testicular tumors may be classified as non-germ cell tumors. This review covers the presentation, diagnosis, and treatment of these rare lesions.     

Quiste epidermoide testicular

IntroductionTesticular tumors are usually managed by radical orchiectomy because of the high incidence of malignant lesions.Epidermoid cyst of the testis is a rare benign tumor, and its differential diagnosis from malignant testicular tumors is difficult.Materials and methodsThe clinical records of seven patients who attended our hospital with testicular epidermoid cysts were reviewed.Preoperative evaluation consisted of testicular ultrasonography in 6 patients, and magnetic resonance imaging in 4 patients. A peroperative biopsy was performed in four patients.ResultsUltrasonographic appearance was specific for diagnosis of epidermoid cyst in 80% of patients. Pathological diagnosis was made in all biopsies taken during surgery.Conservative management was (tumorectomy or partial orquidectomy) performed in 6 patients (85%).ConclusionsPreoperative imaging findings, gross characteristics of the lesion, and peroperative biopsy results provide adequate information to attempt testis-sparing surgery instead of radical orchiectomy.