ABSTRACT
Surgery is the cornerstone of treatment for penile squamous cell carcinoma. Following surgical excision, reconstructive surgery is beneficial to restore aesthetics, functionality, and overall quality of life of these patients. In this mini-review, we discuss the use of skin grafts, perineal urethrostomy, phalloplasty, and vascularised flaps as reconstructive options following penile cancer treatment. Illustrated by videos, we highlight the surgical approach, indications, complications, and outcomes of these reconstructive strategies. PATIENT SUMMARY: Reconstructive surgery is important to restore appearance, urinary function, and sexual function in patients who have been treated for penile cancer. We discuss the benefits and potential complications of various surgical reconstructive options, which are illustrated with videos.
ABSTRACT
BACKGROUND: Nipple preservation contributes to aesthetic outcome and quality of life in women undergoing Skin-Sparing Mastectomy (SSM) with immediate breast reconstruction for the treatment of breast cancer. Intraoperative Frozen Section (IFS) has been advocated to facilitate conversion from Nipple-Sparing Mastectomy (NSM) to SSM in cases with positive subareolar margins. This study investigated the application of IFS at our comprehensive cancer centre. METHODS: In this single-centre retrospective study, for all patients who underwent therapeutic NSM with IFS from 2000 to 2021 pathological reports, patient- and tumour characteristics were retrieved. RESULTS: In total 640 women were included in whom 662 intended NSMs with IFS had been performed. Sensitivity and specificity of frozen section compared with definitive histopathology were 75.2% and 98.5% respectively. In six women with a false positive result, the nipple had been removed. In 16 out of 32 women with a false negative result, the nipple was excised in a second procedure. In total 115 nipples were resected. In 40% of these nipples, no residual disease was detected. DISCUSSION: IFS is a moderately sensitive and highly specific diagnostic tool to detect positive subareolar margins. An alternative approach is to omit frozen section but take intraoperative biopsies of the sub areolar margin, which are postoperatively analysed with definitive formalin-fixed paraffin-embedded histopathology. This allows for shared decision making regarding nipple excision in cases where minimal disease is found in subareolar tissue or cases with an indication for post-mastectomy radiotherapy.
Subject(s)
Breast Neoplasms , Frozen Sections , Nipples , Humans , Female , Breast Neoplasms/surgery , Breast Neoplasms/pathology , Retrospective Studies , Nipples/surgery , Nipples/pathology , Middle Aged , Adult , Aged , Mastectomy, Subcutaneous/methods , Organ Sparing Treatments/methods , Margins of Excision , Mammaplasty/methods , Sensitivity and Specificity , Intraoperative Care/methodsABSTRACT
PURPOSE: This observational study aims to assess the feasibility of calculating indicators developed by the European Commission Initiative on Breast Cancer (ECIBC) for the Dutch breast cancer population. METHODS: Patients diagnosed with invasive or in situ breast cancer between 2012 and 2018 were selected from the Netherlands Cancer Registry (NCR). Outcomes of the quality indicators (QI) were presented as mean scores and were compared to a stated norm. Variation between hospitals was assessed by standard deviations and funnel plots and trends over time were evaluated. The quality indicator calculator (QIC) was validated by comparing these outcomes with the outcomes of constructed algorithms in Stata. RESULTS: In total, 133,527 patients were included. Data for 24 out of 26 QIs were available in the NCR. For 67% and 67% of the QIs, a mean score above the norm and low or medium hospital variation was observed, respectively. The proportion of patients undergoing a breast reconstruction or neoadjuvant systemic therapy increased over time. The proportion treated within 4 weeks from diagnosis, having >10 lymph nodes removed or estrogen negative breast cancer who underwent adjuvant chemotherapy decreased. The outcomes of the constructed algorithms in this study and the QIC showed 100% similarity. CONCLUSION: Data from the NCR could be used for the calculation of more than 92% of the ECIBC indicators. The quality of breast cancer care in the Netherlands is high, as more than half of the QIs already score above the norm and medium hospital variation was observed. The QIC can be easy and reliably applied.
Subject(s)
Breast Carcinoma In Situ , Breast Neoplasms , Humans , Female , Quality Indicators, Health Care , Breast Neoplasms/diagnosis , Breast Neoplasms/epidemiology , Breast Neoplasms/therapy , Netherlands/epidemiology , HospitalsABSTRACT
BACKGROUND: COVID-19 has impacted breast implant surgery for oncological and non-oncological patients worldwide. This population-based study aimed to evaluate the impact of the COVID-19 pandemic on access to reconstructive and cosmetic breast implant surgery in the Netherlands using real-world data to describe trends, and to identify lessons to prevent future capacity problems within (inter)national healthcare. METHODS: This longitudinal study included patients undergoing breast implant surgery from the mandatory nationwide Dutch Breast Implant Registry. For 2020, the first COVID-19 wave, intermediate period, and second wave were defined. We compared data from during the pandemic to a pre-pandemic (2019) reference year, assessing differences in the number of registered breast implants, and patient and surgery-related characteristics. RESULTS: A total of 34133 breast implants (17459 patients) were included. Compared to 2019, fewer implants were registered for post-cancer (n=484; -14.7%), cosmetic (n=480; -3.6%), and gender-affirming indications (n=104; -38.0%) during 2020. Fewer implants were registered in academic (n=196; -22.0%) and regional hospitals (n=1591; -16.5%), but more in private clinics (n=725; +10.1%). After the first wave, up to twice as many implants were registered in private clinics compared to 2019. No differences were found in characteristics of patients undergoing surgery in 2020 versus 2019. CONCLUSION: Hospital-based reconstructive and gender-affirming surgery were heavily impacted during the pandemic, while private-clinic-based cosmetic surgery quickly recovered. These outcomes are useful to fuel discussions about how healthcare could be reorganized in times of capacity problems. We suggest exploring options to deploy private clinics for ambulatory surgery aiming to keep hospital capacity available for acutely ill patients.
Subject(s)
Breast Implantation , Breast Implants , Breast Neoplasms , COVID-19 , Mammaplasty , Humans , Female , Longitudinal Studies , Pandemics , COVID-19/epidemiology , Breast Neoplasms/surgery , Breast Neoplasms/epidemiology , RegistriesABSTRACT
Introduction: The NABON Breast Cancer Audit showed that more than 70% of the Dutch women undergoing surgery for breast cancer maintained their breast contour by breast-conserving surgery (BCS) or by immediate reconstruction after ablative surgery. The proportion of oncoplastic surgery applied in patients undergoing breast-conserving treatment remains unknown. The aim of our study was to assess the need for standardization of oncoplastic breast-conserving surgery (OPBCS) in an attempt to enable measurement of the quality of OPBCS. Methods: To gain a better understanding of current practice in OPBCS, we sent a questionnaire to all breast surgeons in The Netherlands who are members of the breast surgery working group (n = 134). Results: A total of 60 breast surgeons, representing different hospitals in The Netherlands, responded. 61.7% of the breast surgeons performed BCS on 60-100% of their patients. 68.3% responded that BCS was performed using OPS techniques in up to 40% of their patients. OPBCS was defined as level I volume displacement by 45.2% of the breast surgeons and as BCS performed by a breast surgeon and plastic surgeon together by 32.3% of the breast surgeons. 94.5% indicated that there is a need for standardization of the definition of OPBCS in The Netherlands. Conclusion: This study demonstrates that OPBCS is a major part of daily clinical practice of Dutch breast surgeons treating BC patients. Despite this, there is no clear definition of OPS in breast-conserving treatment in The Netherlands. Only after standardization can a classification code and quality indicator be initiated for OPBCS. Ultimately, this will facilitate improvement in quality of BC care.
ABSTRACT
PURPOSE: Oncoplastic surgery (OPS) after breast conserving surgery is preferably performed during the same operation. Offering delayed OPS instead of mastectomy to patients with a high risk of tumor-positive margins allows breast conservation with the option of margin re-excision during OPS, without having to dismantle the reconstruction. We aimed to evaluate surgical outcomes after immediate and delayed OPS. METHODS: We included early-stage breast cancer patients who underwent OPS at the Netherlands Cancer Institute between 2016 and 2019. Patients were selected for delayed OPS after multidisciplinary consultation if the risk of tumor-positive margins with immediate OPS was considered significant (> 30%). Groups were compared on baseline characteristics and short-term surgical outcomes. RESULTS: Of 242 patients with 251 OPS, 130 (52%) OPS had neoadjuvant chemotherapy. Immediate OPS was performed in 176 (70%) cases and delayed OPS in 76 (30%). Selection for delayed OPS was associated with tumor size (OR 1.03, 95% CI 1.01-1.04), ILC (OR 2.61, 95% CI 1.10-6.20), DCIS (OR 3.45, 95% CI 1.42-8.34) and bra size (OR 0.76, 95% CI 0.62-0.94). Delayed and immediate OPS differed in tissue weight (54 vs. 67 g, p = 0.034), tissue replacement (51% vs. 26%, p < .001) and tumor-positive margins (66% vs. 18%, p < .001). Re-excision was performed in 48 (63%) delayed OPS and in 11 (6%) immediate OPS. Groups did not differ in complications (21% vs. 18%, p = 0.333). Breast conservation after immediate and delayed OPS was 98% and 93%, respectively. CONCLUSION: Performing delayed OPS in selected cases facilitated simultaneous margin re-excision without increasing complications, and resulted in an excellent breast conservation rate.
Subject(s)
Breast Neoplasms , Mammaplasty , Humans , Female , Breast Neoplasms/surgery , Breast Neoplasms/pathology , Mastectomy, Segmental/adverse effects , Mastectomy, Segmental/methods , Cohort Studies , Mastectomy/adverse effects , Netherlands/epidemiology , Mammaplasty/methods , Retrospective StudiesABSTRACT
Primary intra- or transabdominal transplantation of an extended rectus abdominis myocutaneous (ERAM) flap may help prevent surgical complications of pelvic resections. Surgical delay of the ERAM flap may help prevent intra-abdominal (partial) flap loss after transplantation in highly complex situations including previous irradiation. We report on the outcome of this approach and the risk-factors associated with an eventful outcome. From 2012 to 2020, 105 delayed ERAM flaps were consecutively applied immediately following extended pelvic resections after chemoradiation or hyperthermic intraperitoneal chemotherapy. We addressed the increased reconstructive demands by designing the flap in line with the 10th rib and delaying the flap's skin island. All post-operative complications were assessed in light of patient-related or procedure-related potential risk-factors. Major complications occurred in 39 patients. These were correlated with surgery for residual or recurrent malignancy (p < 0.01), with tip necrosis after flap delay (p = 0.02), and with the use of a mesh to close the abdominal donor site (p < 0.01). (Partial) flap loss occurred in 4 cases. We observed a comparably high rate of major complications after ERAM transplantations for increasingly extending indications of perineal-pelvic resections. We consider this to be attributable to poorer patients' conditions and disease processes, rather than to flap viability. Delay of the flap allowed for the use of large and voluminous flaps with comparably little (partial) flap loss.
Subject(s)
Myocutaneous Flap , Plastic Surgery Procedures , Humans , Myocutaneous Flap/surgery , Rectus Abdominis/transplantation , Neoplasm Recurrence, Local/complications , Neoplasm Recurrence, Local/surgery , Abdominal Muscles/surgery , Postoperative Complications/surgeryABSTRACT
Autologous breast reconstructions are increasingly being performed. While increased risks for flap loss after free tissue transfer (FTT) in sickle cell patients are known to most plastic surgeons, risks for patients with sickle cell trait (SCT) are less known. Unfortunately, recommendations to prevent complications after FTT in SCT are still scarce. We present additional and novel surgical measures that we took aiming to enhance probabilities for successful FTT in a SCT patient undergoing autologous breast reconstruction. This is only the second case report describing a successful FTT without flap necrosis/loss in a patient with SCT, and the first successful report involving FTT in the breast.
Subject(s)
Anticoagulants/adverse effects , Free Tissue Flaps , Hematoma/chemically induced , Heparin/adverse effects , Mammaplasty/methods , Postoperative Hemorrhage/etiology , Sickle Cell Trait/complications , Female , Free Tissue Flaps/blood supply , Free Tissue Flaps/transplantation , Graft Survival , Heparin/administration & dosage , Humans , Mammaplasty/adverse effects , Middle Aged , Treatment OutcomeABSTRACT
BACKGROUND: Breast-conserving therapy is defined as a breast-conserving wide local excision (WLE) of a mammary tumour combined with postoperative radiotherapy. Immediate restoration of the mammary shape by use of breast reduction techniques (volume displacement) or tissue replacement techniques (volume replacement) is gaining popularity to prevent breast malformation. METHODS: To date, using the internal mammary artery perforator (IMAP) flap has been suggested for immediate volume replacement after WLE, but has never been evaluated in a published study. RESULTS: We applied this flap in 12 women (mean age, 56.1 years) after WLE (mean specimen weight, 46.5 g) of the medial aspect of the breast. Over a median follow-up of 35.3 months (standard deviation, 1.2 months), 4 women needed repeated surgery for dog-ear correction of the donor site. CONCLUSIONS: In our experience, the use of an IMAP flap was a reliable technique with good cosmetic outcomes after oncoplastic reconstruction. In this series, donor site revision often proved necessary initially, but we showed that this may easily be prevented.
ABSTRACT
An estimated 1-3% of all women in the Netherlands carry breast implants. Since the introduction five decades ago, problems with a variety of breast implants have emerged with direct consequences for the patients' health. Plastic surgeons worldwide reacted through campaigning for auditing on long-term implant quality, surgeon performance, and institutional outcomes in implant registries. Especially, the PIP implant scandal of 2010 demonstrated the paucity of epidemiological data and uncovered a weakness in our ability to even 'track and trace' patients. In addition, a recent report of the Dutch Institute of National Health showed a lack of compliance of 100% of breast implant producers to CE requirements. These arguments stress the need for an independent implant registry. Insufficient capture rates or dependence from the implant producers made the variety of national and international patient registries unreliable. The Dutch Breast Implant Registry (DBIR) is unique because it is an opt-out registry without the need for informed consent and thus a high capture rate. Furthermore, an estimated 95% of breast implants are implanted by board-certified plastic surgeons. Funding was received from a non-governmental organisation to increase the quality of health care in the Netherlands, and maintenance is gathered by 25 euros per implant inserted. This article describes the way the Dutch have set up their system, with special attention to the well-known hurdles of starting a patient registry. Examples include: funding, medical ethical issues, opt out system, benchmarking, quality assurance as well as governance and collaboration. The Dutch consider their experience and data shareware for others to be used globally to the benefit of patient safety and quality improvement.
Subject(s)
Breast Implantation , Breast Implants , Quality of Health Care/standards , Breast Implantation/adverse effects , Breast Implantation/methods , Breast Implantation/statistics & numerical data , Breast Implants/adverse effects , Breast Implants/statistics & numerical data , Commission on Professional and Hospital Activities/organization & administration , Female , Humans , Needs Assessment , Netherlands , Organizational Innovation , Patient Safety/standards , Prosthesis Failure , Quality Improvement , RegistriesABSTRACT
OBJECTIVE: Dissection of the latissimus dorsi (LD) flap may have a distinct impact on upper extremity function and strength. To date, insufficient differentiation has been made between loss of muscular strength and shoulder function by dissection of the LD muscle per se and loss of function resulting from all excisional surgery, radiation therapy, and reconstructive procedures at the donor site and the recipient site that may have been combined near the shoulder region. METHODS: This study determines the long-term effect of the LD breast reconstruction on the strength profiles of the upper extremity by measuring the isometric torque strength both in seven synergistic- and two contra-movement directions. The Biodex System 3 Pro (Biodex Medical Systems, New York, NY) was used in 12 patients at a mean of 3.5 years after surgery, and in 20 matched controls. Because loss of LD muscle may not result in significant impairment of activities of daily live (ADL) even in cases where objective measurements are decreased, this study simultaneously assessed the subjective function by use of the standardised Disability of Arm, Shoulder and Hand (DASH) questionnaire. RESULTS: This study observed a significant long-term loss of 8.8 Nm or 19% LD torque strength in synergistic movement directions after transplantation of the LD muscle. This loss correlated significantly with an increase of the mean DASH score among the patients. CONCLUSIONS: Because no significant loss of contra-movement torque strength was observed, it was concluded that the loss of synergistic torque strength appears to result from the loss of LD function per se.
Subject(s)
Mammaplasty/adverse effects , Muscle Strength , Superficial Back Muscles/transplantation , Upper Extremity/physiopathology , Adult , Aged , Breast Neoplasms/radiotherapy , Breast Neoplasms/surgery , Case-Control Studies , Female , Humans , Mammaplasty/methods , Middle Aged , Postoperative Complications/physiopathology , Surgical Flaps , Surveys and Questionnaires , Upper Extremity/physiologyABSTRACT
BACKGROUND: Local relapse after breast-conserving therapy including whole breast irradiation is typically treated by salvage mastectomy. Immediate reconstruction by pedicled transfer of a latissimus dorsi flap in combination with implantation of a definitive prosthesis or temporary tissue expander following skin sparing salvage mastectomy has been shown to be feasible. However, it has never been shown to be justifiable. AIM: The aim of the study was to compare the outcome of this procedure to the widely accepted secondary breast reconstruction by combined latissimus dorsi flap and implant after mastectomy and adjuvant radiotherapy. METHODOLOGY: The surgical outcome of 93 immediate latissimus dorsi and implant reconstructions after skin-sparing salvage mastectomy performed from 2007 to 2011 after radiotherapy was compared to that of 83 secondary reconstructions with the latissimus dorsi and an implant. The follow-up duration was 3.5 years in both groups. Complications were categorized as minor (conservative treatment sufficed) or major (flap loss, mammary skin loss, implant loss, seroma or haematoma indicating repeat surgery). RESULTS: The salvage group scored significantly less on half of the patient-related and procedure-related risk factors. Nevertheless, we observed 27% of short-term major surgical complications and an ultimate success rate of 94% in the salvage group compared to those observed in our series of secondary reconstruction in post-radiation women (27% and 93%, respectively). CONCLUSION: Skin-sparing salvage mastectomy combined with immediate reconstruction by transfer of a latissimus dorsi flap with an implant is a justifiable reconstructive option for women with a recurrence after irradiation as part of breast-conserving therapy.
Subject(s)
Breast Implants , Breast Neoplasms/surgery , Mammaplasty , Mastectomy, Segmental , Myocutaneous Flap , Salvage Therapy , Adult , Aged , Breast Neoplasms/pathology , Breast Neoplasms/radiotherapy , Female , Humans , Middle Aged , Radiotherapy, Adjuvant , Reoperation , Superficial Back Muscles , Time Factors , Young AdultABSTRACT
BACKGROUND AND AIM: Early age malignant tumors can mimic benign infantile hemangioma (IH). The purpose of this study was to determine the percentage of patients referred with a diagnosis of IH that were misdiagnosed, including that of those who in fact had malignant lesions and to assess how the misdiagnosis was reached. METHODS: From April 2003 through December 2009, all records of children, referred with a diagnosis of IH, were studied to determine the definitive diagnosis. Characteristics of the children with malignant diagnoses and of their diagnostic process were retrospectively analyzed. RESULTS: The referral diagnosis "hemangioma" was recorded in 423 children and confirmed in 89%. Thirty-nine children were rediagnosed with a vascular malformation (7%) or other benign anomalies (2%). The 7 (2%) remaining children were diagnosed with a malignancy. CONCLUSIONS: In cases where the diagnosis of IH is equivocal, biopsy has to be performed in a specialized center to prevent delay.
Subject(s)
Diagnostic Errors/statistics & numerical data , Hemangioma, Capillary/diagnosis , Skin Neoplasms/diagnosis , Soft Tissue Neoplasms/diagnosis , Adolescent , Child , Child, Preschool , Delayed Diagnosis/statistics & numerical data , Diagnosis, Differential , Female , Humans , Infant , Infant, Newborn , Male , Vascular Malformations/diagnosisABSTRACT
PURPOSE: To compare intralesional corticosteroid (IC) injections with oral propranolol in children with periorbital infantile hemangioma (IH). METHODS: Children were diagnosed with IH and treated with IC (n = 29) or propranolol (n = 14). In cases in which no further improvement was seen, treatment was stopped and the outcome measured. Type, duration, and complications of treatment and supportive or additional therapies were scored, along with outcomes of visual measurements, at the start and end of therapy. RESULTS: Twenty-one of the 29 children (72%) treated with IC injections were given a second injection and 16 (55%) were given more than two. Median duration of IC therapy was 15.9 months (interquartile range (IQR) 10.28), compared with 6.5 months (IQR 4.87) for propranolol (p<0.001). The complications reported after IC injections were bleeding (n = 9) and ulceration (n = 7). No complications were noted in the propranolol group. Additional therapy consisting of oral prednisolone therapy was applied in one child from the steroid-treated group. The levels of amblyopia and the median absolute improvement did not significantly differ between the groups. CONCLUSIONS: The IC injections and oral propranolol medication equally improved amblyopia in children with IH of the periorbital and cheek region; however, propranolol was associated with fewer complications and additional or supportive treatment was not indicated. We prefer oral propranolol medication over IC injections in cases in which IH threatens to hamper visual acuity.
Subject(s)
Adrenergic beta-Antagonists/administration & dosage , Eyelid Neoplasms/drug therapy , Glucocorticoids/administration & dosage , Hemangioma/drug therapy , Prednisolone/administration & dosage , Propranolol/administration & dosage , Skin Neoplasms/drug therapy , Administration, Oral , Child , Child, Preschool , Eyelid Neoplasms/physiopathology , Female , Hemangioma/physiopathology , Humans , Infant , Injections, Intralesional , Male , Skin Neoplasms/physiopathology , Treatment Outcome , Visual Acuity/physiologyABSTRACT
Haemangiomas are considered to be the most common tumours of infancy. However, despite their frequent occurrence the aetiological determinants of their development remain unknown. Identifying these factors may provide insight on their pathogenesis. We performed cross-sectional screening for haemangiomas in newborns (0-16 months of age) in the general Dutch population. Haemangioma-specific and pregnancy-related data were collected in children with a haemangioma. These data were compared in a case-control design using multivariable logistic regression analysis. Among 2204 newborns, 219 (9.9%) had a haemangioma. The male-to-female ratio was 1:2. The majority of haemangiomas were located on the trunk (37%). The general practitioners or medical specialists were consulted in only a minority of cases (<7%). Amniocentesis [odds ratio (OR) 3.6, 95% confidence interval (CI) 1.11, 11.42], breech presentation [OR 2.3, 95% CI 1.14, 4.44], being first-born [OR 1.55, 95% CI 1.03, 2.33] and a birthweight ≤2500 g [OR 4.95, 95% CI 1.63, 15.05] were independent factors associated with the development of a haemangioma. Duration of pregnancy did not differ between study groups. Our study showed that the prevalence of a haemangioma is 9.9% in the general (Dutch) population. Four factors appear relevant in the development of haemangiomas. These factors may provide clues to its pathogenesis.
Subject(s)
Hemangioma/epidemiology , Pregnancy Complications, Neoplastic/epidemiology , Amniocentesis/adverse effects , Birth Order , Birth Weight , Breech Presentation , Case-Control Studies , Female , Hemangioma/etiology , Humans , Infant , Logistic Models , Male , Netherlands/epidemiology , Pregnancy , Pregnancy Complications, Neoplastic/etiology , Prevalence , Risk Factors , Sex FactorsABSTRACT
BACKGROUND: Haemangiomas are the most common tumours of infancy, they are often present on the face and can lead to disfigurement. The aim of our study was to assess the health-related quality of life (HRQoL) of children aged 1-15 years with a haemangioma and their parents in comparison with healthy children, and to assess the impact on their life. Furthermore, we investigated if visibility and a complicated course of the haemangioma had influence on these outcomes. METHODS: Age-specific validated HRQoL and haemangioma-specific questionnaires were sent to all children seen at the AMC in Amsterdam at the plastic surgery or dermatology departments. RESULTS: Two hundred and one parents of children with a haemangioma returned the questionnaire (85%). The majority of parents and patients with a haemangioma are not negatively affected by it. Concerning HRQoL, parents of children aged 8 to 11 years reported their children to have more negative emotions. Children aged 12 to 15 reported a better HRQoL for physical symptoms, and positive emotions. No differences were found in the HRQoL questionnaires between visibility and complicated course. Significant differences occur in the specific haemangioma questionnaire. High scores on feelings of disbelief and panic during the growing phase are given by parents and patients agree with the statement on whether their life would be different without a haemangioma, especially when the haemangioma was visible and/or had a complicated course. CONCLUSIONS: This is the first study to describe the HRQoL of children with haemangiomas. The psychosocial consequences of a haemangioma are feared by physicians and parents. The most important finding of this study is that the majority of children of all ages and their parents feel children with haemangiomas can live a good life. There is reason, however, to believe that having a haemangioma, especially one with a complicated course or a visible location, could result in psychosocial problems later in life, mostly related to physical appearance.
Subject(s)
Hemangioma/psychology , Parents/psychology , Quality of Life/psychology , Surveys and Questionnaires , Adolescent , Child , Humans , Stress, PsychologicalABSTRACT
This case report presents an incidental finding of a rectal GIST (gastrointestinal stromal tumor) presenting as a submucosal calculus, not previously reported. A 53-year-old man without a significant medical history presented with abdominal pain in the left lower quadrant, and with constipation. Upon rectal examination, a hard submucosal swelling was palpated 4 cm from the anus, at 3 o'clock, in the left rectum wall. X-ray photos, computerized tomography (CT)-scan and a magnetic resonance imaging (MRI) scan clearly showed a calculus. Excision revealed a turnip-like lesion, 3.1 x 2.3 x 1.8 cm. Analysis showed it was a rectal GIST, a rare mesenchymal tumor of the gastrointestinal tract, which expressed CD117 (or c-kit, a marker of kit-receptor tyrosine kinase) and CD34. Calcification is not a usual clinicopathological feature of GISTs [1-3], and although a number of rectal GISTs have been reported [4-9], we have found no cases so far of rectal GIST presenting as a submucosal calculus. In general, GISTs are rare mesenchymal tumors of the gastrointestinal tract (nerve tissue, smooth muscle). Histology and immunohistochemistry discriminate gastrointestinal stromal tumors from leiomyomas and neurinomas. The most important location is the stomach; the rectal location is rare. Usually, the classic signs of malignancy such as cellular invasion and metastasis are missing. A set of histologic criteria stratifies GIST for risk of malignant behavior such as mitotic activity and tumor size, cellular pleomorphism, developmental stage of the cell and quantity of cytoplasma [7,13]. Tumors with a high mitotic activity and size above 5 cm are considered malignant. Recent pharmacological advances such as tyrosine kinase inhibitors have determined c-kit (i.e., CD117) as the most important marker, amongst others. C-kit positive tumors respond extremely well to chemotherapy with Imatinib (Glivec, Gleevec) [10-12].