ABSTRACT
BACKGROUND: Papillary thyroid carcinoma (PTC) is a common neoplasia with multiple variants. One of these extremely rare and poorly described variants is PTC with fibromatosis-like stroma (PTC-FMS), a peculiar entity distinguished by its predominant mesenchymal component. This paper reviews the literature, discusses the diagnostic challenges, and the clinical and surgical implications of this type of tumor which has fewer than 30 cases reported in the literature. CASE PRESENTATION: We reported a case of PTC-FMS found in a 41-year-old Italian woman, who came to our Institute with a recent growth in the form of a mass on the neck. Further immunohistochemical examination showed ß-catenin aberrant staining both in the nuclei and cytoplasm of the mesenchymal cells. The patient underwent total thyroidectomy and received radioactive iodine (RAI) 2 months after surgery. CONCLUSION: Given the possibility of recurrence of PTC-FMS and the ineffectiveness of RAI therapy, complete surgical resection represents the main treatment for this type of tumor. Despite the fact that the specific nature of these lesions has yet to be determined, guidelines for classical PTC should be followed.
Subject(s)
Carcinoma, Papillary , Fibroma , Thyroid Neoplasms , Female , Humans , Adult , Thyroid Cancer, Papillary/diagnosis , Thyroid Cancer, Papillary/surgery , Thyroid Neoplasms/diagnosis , Thyroid Neoplasms/surgery , Thyroid Neoplasms/pathology , Iodine Radioisotopes/therapeutic use , Carcinoma, Papillary/diagnosis , Carcinoma, Papillary/surgery , Carcinoma, Papillary/pathology , Thyroidectomy , Fibroma/drug therapy , Fibroma/surgeryABSTRACT
RATIONALE: Pachydermodactyly is a rare, benign disease that can manifest in healthy adolescent boys as painless, spindle-shaped, soft-tissue swelling of the proximal interphalangeal joints in the hand. It is usually bilateral, with symmetrical joint enlargement. There are relatively few documented cases of pachydermodactyly worldwide, signifying either a low incidence or lack of recognition by physicians; therefore, its diagnosis is challenging. PATIENT CONCERNS: A 16-year-old boy with a 3-year history of painless unilateral swelling of the proximal interphalangeal joints of his left hand was misdiagnosed with juvenile idiopathic arthritis and was treated with oral methotrexate for 1 year. He had a history of frequent finger cracking. DIAGNOSIS: He had normal levels of inflammatory markers, including erythrocyte sedimentation rate and C-reactive protein. His autoantibody profile results were normal, and radiography of his hands showed soft tissue swelling with no bone abnormalities. Therefore, the patient was diagnosed with Parkinson disease. INTERVENTIONS: Methotrexate was discontinued, and a skin biopsy was performed, which revealed hyperkeratosis in the epidermis with thick collagenous fibers in the dermis. Therefore, the patient was informed of the benign nature of the disease and was advised to stop cracking his fingers. OUTCOMES: After regular follow-up, there was no progression of the patient's symptoms, and repeated blood tests revealed normal results. LESSONS: Pachydermodactyly should be considered in the differential diagnosis of painless swelling in adolescent men with normal blood testing. Early recognition of this rare benign condition helps physicians appropriately reassure the patient and his parents without exposing them to unnecessary therapy.
Subject(s)
Arthritis, Juvenile , Fibroma/congenital , Fingers/abnormalities , Adolescent , Arthritis, Juvenile/diagnosis , Arthritis, Juvenile/drug therapy , Diagnostic Errors , Edema , Fibroma/diagnosis , Fibroma/drug therapy , Hand , Humans , Male , Methotrexate/therapeutic useABSTRACT
INTRODUCTION: Pazopanib, a tyrosine kinase inhibitor (TKI), is a standard treatment for various tumours, including metastatic non-adipocytic soft-tissue sarcomas. In literature, erythrocytosis has been described as a TKI-related condition. CASE REPORT: A 59-year-old man underwent surgical removal of a sub-scapular mass consistent with myxofibrosarcoma. After distant relapse, he first started chemotherapy, and then Pazopanib. He was found to have increased levels of hemoglobin (Hb) and hematocrit (Hct). He was asymphtomatic, with no history of pulmonary disease nor smoking habit. Erythropoietin (EPO) level was higher than normal. A polycythemia vera was ruled out.Management & outcome: The patient started a prophylactic therapy with lysine acetylsalicylate, and we observed a reduction of Hb, but not Hct. Due to disease progression, we interrupted Pazopanib. After a week from drug discontinuation, Hb levels got back to the normal range, Hct was lowering. We decided not to perform phlebotomy, considering the declining trend in Hb and Hct values and the absence of symptoms. DISCUSSION: We postulated a Pazopanib-related secondary erythrocytosis, since Hb and Hct levels increased from baseline during treatment, then normalized when Pazopanib was discontinued. We used the Naranjo Nomogram to assess the correlation between the adverse effect and Pazopanib, the correlation was "Probable", a score of 5. To the best of our knowledge, this is the first case report of Pazopanib-related secondary polycythemia in a patient with sarcoma. It is important to pay attention to blood count and to any symptoms potentially related to erythrocytosis in patients treated with TKIs.
Subject(s)
Fibroma/drug therapy , Fibrosarcoma/drug therapy , Polycythemia/chemically induced , Protein Kinase Inhibitors/adverse effects , Pyrimidines/adverse effects , Sulfonamides/adverse effects , Angiogenesis Inhibitors/adverse effects , Fibroma/diagnosis , Fibrosarcoma/diagnosis , Humans , Indazoles , Male , Middle Aged , Polycythemia/diagnosis , Soft Tissue Neoplasms/drug therapyABSTRACT
BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype with a generally indolent pattern of clinical behaviour, but treatments for advanced disease are limited. PATIENTS AND METHODS: A retrospective search of a prospectively maintained institutional database identified 102 patients treated from December 1994 to August 2018. We evaluated the outcome of patients and the efficacy and safety of non-surgical therapies in LGFMS. RESULTS: Ninety-four out of 102 (92.2%) underwent primary resection, seven (6.9%) were treated with systemic therapy and one (1.0%) is currently being treated with pre-operative radiotherapy. The RECIST 1.1 response rate to first-line chemotherapy was 0%, and median progression-free survival was 1.84 months (95% confidence intervaI=0.10-3.6 months). CONCLUSION: Conventional systemic therapy has limited efficacy in advanced LGFMS.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Fibroma/drug therapy , Fibrosarcoma/drug therapy , Adult , Combined Modality Therapy , Female , Fibroma/pathology , Fibroma/therapy , Fibrosarcoma/pathology , Fibrosarcoma/therapy , Follow-Up Studies , Humans , Male , Middle Aged , Neoplasm Grading , Prospective Studies , Retrospective Studies , Survival RateSubject(s)
Bird Diseases/drug therapy , Cisplatin/therapeutic use , Cockatoos , Electrochemotherapy/veterinary , Fibroma/veterinary , Skin Neoplasms/veterinary , Animals , Antineoplastic Agents/administration & dosage , Antineoplastic Agents/therapeutic use , Cisplatin/administration & dosage , Fibroma/drug therapy , Male , Skin Neoplasms/drug therapyABSTRACT
Nonislet cell tumor hypoglycemia is rare. We highlight the diagnosis and treatment of recurrent severe hypoglycemia in a 49-year-old woman with malignant solitary fibrous tumor of the pleura (Doege-Potter syndrome). The clinical, laboratory and radiologic findings of the case are presented and a brief literature review is provided. Of note, imaging studies showed a large mass in the right hemithorax and pathology and immunehistochemical stains confirmed a malignant solitary fibrous tumor of the pleura. She was a poor surgical candidate owing to a large tumor burden. She was treated with a combination of temozolomide and bevacizumab to which she responded with resolution of hypoglycemia. The treatment of choice for hypoglycemia in patients with the Doege-Potter syndrome is surgical excision. We here report that a combination of temozolomide and bevacizumab may be a viable option in patients with inoperable disease.
Subject(s)
Bevacizumab/adverse effects , Fibroma , Hypoglycemia/chemically induced , Pleural Neoplasms , Temozolomide/adverse effects , Bevacizumab/administration & dosage , Female , Fibroma/diagnosis , Fibroma/drug therapy , Humans , Middle Aged , Pleural Neoplasms/diagnosis , Pleural Neoplasms/drug therapy , Temozolomide/administration & dosageABSTRACT
Although certainly not the first line treatment for plantar fibromas, surgical resection is a treatment option for some patients with have failed exhaustive non-surgical treatment. The use of topical Mitomycin C has been recently shown to reduce the recurrence rate of other fibrous lesions. The purpose of this study was to determine the impact of topical application of Mitomycin C on recurrence rate of plantar fibromas. A retrospective analysis was done from a prospectively gathered database with a total 50 consecutive patients over a 16-month study period. The control group (n = 29) consisted of patients who underwent only surgical resection, while the study group (n = 21) consisted of patients who underwent surgical resection with adjuvant therapy using Mitomycin C. The primary endpoint was local recurrence after the procedure. Secondary end points included complications and toxicity associated with this medication. No patients were lost to follow up. Of the 29 patients in the control group, there were 17 patients (17/29, 58.6%) had recurrence of the plantar fibroma at a mean follow-up of 9.1 months. In contrast, in the experimental study group, all patients were free from local recurrence. No complications or side effects were associated with Mitomycin C use. The results demonstrate that the topical application of Mitomycin C to the tumor bed after surgical resection of plantar fibromas reduced the recurrence rate. © 2018 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 36:2554-2561, 2018.
Subject(s)
Fibroma/drug therapy , Fibroma/metabolism , Fibroma/surgery , Foot Diseases/drug therapy , Foot Diseases/metabolism , Foot Diseases/surgery , Mitomycin/pharmacology , Adult , Aged , Aged, 80 and over , Female , Follow-Up Studies , Humans , Male , Middle Aged , Prospective Studies , Recurrence , Regression Analysis , Retrospective Studies , Risk Factors , Time Factors , Treatment Outcome , Wound Healing , Young AdultSubject(s)
Adenomatous Polyposis Coli/drug therapy , Antineoplastic Agents/adverse effects , Duodenal Neoplasms/drug therapy , Erythema Nodosum/chemically induced , Fibroma/drug therapy , Neoplasms, Multiple Primary/drug therapy , Pancreatic Neoplasms/drug therapy , Sorafenib/adverse effects , Aged , Erythema Nodosum/diagnosis , Erythema Nodosum/pathology , Female , Humans , Middle AgedABSTRACT
Desmoplasia is a fibro-inflammatory process and a well-established feature of pancreatic cancer. A key contributor to pancreatic cancer desmoplasia is the pancreatic stellate cell. Various in vitro and in vivo methods have emerged for the isolation, characterization, and use of pancreatic stellate cells in models of cancer-associated fibrosis. In addition to cell culture models, genetically engineered animal models have been established that spontaneously develop pancreatic cancer with desmoplasia. These animal models are currently being used for the study of pancreatic cancer pathogenesis and for evaluating therapeutics against pancreatic cancer. Here, we review various in vitro and in vivo models that are being used or have the potential to be used to study desmoplasia in pancreatic cancer.
Subject(s)
Biomedical Research/methods , Disease Models, Animal , Fibroma/etiology , Pancreatic Neoplasms/physiopathology , Animals , Animals, Genetically Modified , Antineoplastic Agents/pharmacology , Antineoplastic Agents/therapeutic use , Biomedical Research/trends , Cell Line, Tumor , Drugs, Investigational/pharmacology , Drugs, Investigational/therapeutic use , Female , Fibroma/drug therapy , Fibroma/immunology , Fibroma/pathology , Fibrosis , Humans , Male , Mice , Neoplasm Transplantation/methods , Neoplasm Transplantation/trends , Pancreatic Neoplasms/drug therapy , Pancreatic Neoplasms/immunology , Pancreatic Neoplasms/pathology , Pancreatic Stellate Cells/drug effects , Pancreatic Stellate Cells/immunology , Pancreatic Stellate Cells/pathology , Pancreatic Stellate Cells/transplantation , Rats , Tumor Cells, Cultured , Xenograft Model Antitumor Assays/methodsSubject(s)
Abdominal Neoplasms/diagnosis , Fibroma/diagnosis , Fibrosarcoma/diagnosis , Myxoma/diagnosis , Abdomen , Abdominal Neoplasms/drug therapy , Abdominal Neoplasms/pathology , Child , Diagnosis, Differential , Dilatation, Pathologic/etiology , Fibroma/drug therapy , Fibroma/pathology , Fibrosarcoma/pathology , Humans , Male , Myxoma/pathologyABSTRACT
Infantile digital fibroma is a rare benign lesion that usually occurs during the first 2 years of life. It can be multiple, but it is usually a single lesion. If it grows large enough it can cause joint deformities or interfere with everyday activities. Microscopically, the neoplastic cells usually have inclusion bodies that are best highlighted with a Masson trichrome stain but can often be seen on hematoxylin-eosin staining. Treatment for this entity is usually watchful waiting because of its ability to spontaneously regress, but excision is recommended if the lesion is symptomatic. More recently, fluorouracil or injectable steroids have shown great promise in inducing regression without the complications that accompany surgery.
Subject(s)
Epidermis/pathology , Fibroma/pathology , Skin Neoplasms/pathology , Actins/metabolism , Desmin/metabolism , Epidermis/drug effects , Epidermis/metabolism , Fibroma/drug therapy , Fibroma/metabolism , Humans , Immunohistochemistry , Infant , Infant, Newborn , Muscle, Smooth/metabolism , Skin Neoplasms/drug therapy , Skin Neoplasms/metabolismABSTRACT
Pachydermodactyly is a rare skin disease, defined as a benign form of digital fibromatosis. It is clinically characterized by painless hypertrophy of the skin around the proximal interphalangeal joints of the fingers, more common in males. This rare condition can affect both hands and is often associated with mechanical injury of the skin. Although there is no specific therapy, cessation of mechanical trauma associated with topical corticosteroid therapy can lead to satisfactory results. We present 3 sporadic cases of classical pachydermodactyly, anatomopathologically confirmed by the presence hyperkeratosis and acanthosis, which responded favorably to topically applied corticosteroids under occlusive dressings.
Subject(s)
Fibroma/diagnosis , Fibroma/drug therapy , Glucocorticoids/therapeutic use , Skin Neoplasms/diagnosis , Skin Neoplasms/drug therapy , Administration, Cutaneous , Adolescent , Agricultural Workers' Diseases/etiology , Child , Cumulative Trauma Disorders/complications , Diagnosis, Differential , Fibroma/congenital , Fibroma/etiology , Fingers/abnormalities , Fingers/pathology , Glucocorticoids/administration & dosage , Hand , Humans , Male , Occlusive Dressings , Skin Neoplasms/etiology , Treatment OutcomeABSTRACT
Familial multiple discoid fibromas is a rare genodermatosis that bears some resemblance to Birt-Hogg-Dubé syndrome but is not associated with mutations in the folliculin (FLCN) gene or systemic manifestations. It is characterized by the development of papules over the face and pinnae early in life. Histological findings are of fibrovascular tumours adjacent to hair follicles without features characteristic of fibrofolliculomas, which have recently been termed discoid fibromas. We present siblings with multiple papules over the face and pinnae that developed in childhood. Histological specimens from both siblings demonstrated discoid fibromas, but with some lesions exhibiting an unusual keloidal-like pattern with thick hyalinized collagen fibres surrounded by plump spindle and histiocyte-like cells. FLCN gene mutations were not found. We report on clinical improvement with topical rapamycin solution (1 mg mL(-1)) applied daily to the face for 4 months. Therapeutic response to topical rapamycin may provide a clue to the underlying genetic basis of this condition.
Subject(s)
Antibiotics, Antineoplastic/administration & dosage , Facial Neoplasms/drug therapy , Fibroma/drug therapy , Sirolimus/administration & dosage , Skin Neoplasms/drug therapy , Administration, Cutaneous , Adult , Facial Neoplasms/genetics , Facial Neoplasms/pathology , Female , Fibroma/genetics , Fibroma/pathology , Humans , Male , Skin Neoplasms/genetics , Skin Neoplasms/pathology , Treatment Outcome , Young AdultABSTRACT
BACKGROUND/PURPOSE: Fibrous soft tissue tumors (FSTT) of infancy and childhood present a challenge to surgeons due to ambiguous histological interpretation, propensity for local recurrence, and uncertainty regarding appropriate therapy. METHODS: Records were reviewed for patients diagnosed and treated for FSTT at our institution from 1992 to 2010. Data included demographics, tumor location, operative treatment, histopathology, margin status, mitotic index, and recurrence. RESULTS: Eighty-six patients underwent treatment of FSTT. Mean follow-up time was 62 months (range 1 to 201 months). There were 11 recurrences (13%), with a mean time to recurrence of 23 months. Extremity and digital lesions were most likely to recur with 21% of such lesions recurring compared to 7% of lesions located elsewhere. Recurrences were seen more frequently in older children. Despite the presence of positive margins in 92% of cases, margin status, histopathology, and mitotic index were not significant predictors of recurrence. CONCLUSIONS: Digital and extremity fibrous soft tissue tumors and tumors of older children have an increased risk for local recurrence. Margin status did not predict recurrence rates in this series. While the surgical goal is to obtain complete local resection with clear margins, this may be challenging in particular locations and may not ultimately impact recurrence.
Subject(s)
Fibroma/surgery , Neoplasm Recurrence, Local/etiology , Soft Tissue Neoplasms/surgery , Adolescent , Antineoplastic Agents/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Carboplatin/administration & dosage , Chemotherapy, Adjuvant , Child , Child, Preschool , Female , Fibroma/drug therapy , Fibroma/pathology , Follow-Up Studies , Humans , Infant , Infant, Newborn , Logistic Models , Male , Methotrexate/administration & dosage , Retrospective Studies , Risk Factors , Soft Tissue Neoplasms/drug therapy , Soft Tissue Neoplasms/pathology , Treatment Outcome , Vinblastine/administration & dosage , Young AdultABSTRACT
OBJECTIVE: To describe the clinical management of menorrhagia in a woman with hyperparathyroidism-jaw tumor syndrome (HPT-JT). DESIGN: Case report. SETTING: Large translation research hospital. PATIENT(S): A 26-year-old nulligravid woman with familial HPT-JT presented with life-long menorrhagia resistant to progesterone intrauterine device (IUD) therapy and a desire for fertility. INTERVENTION(S): Aromatase inhibitor therapy. MAIN OUTCOME MEASURE(S): Clinical response to therapy and pregnancy. RESULT(S): Imaging demonstrated an enlarged endometrial lining and thickening of the junctional zone. At operative hysteroscopy, multiple atypical endometrial polyp-like lesions filled the entire uterine cavity and were removed. Histologic evaluation demonstrated the lesions to be adenomyomas with an abundance of aromatase expression. Postoperative treatment included an aromatase inhibitor. The patient's menorrhagia, which had previously been resistant to progesterone IUD therapy, resolved with the aromatase inhibitor. After 10 months of this treatment, the aromatase inhibitor was discontinued and a repeated hysteroscopy revealed a markedly improved uterine cavity. The patient subsequently became pregnant on her first natural cycle and delivered a healthy term infant. CONCLUSION(S): Aromatase inhibitors may represent a novel treatment for benign uterine pathology in HPT-JT.