Alternative Lymphatic Drainage Pathways in the Trunk Following Oncologic Therapy.

BACKGROUND: Anatomic and functional descriptions of trunk and breast lymphedema following breast cancer treatment are emerging as indicators of lymphatic dysfunction. Indocyanine green-lymphangiography has been instrumental in characterizing this dysfunction in the extremity and can be applied to other regions. Previous work has established a validated Pittsburgh Trunk Lymphedema Staging System to characterize such affected areas. This study aims to identify risk and protective factors for the development of truncal and upper extremity lymphedema using alternative lymphatic drainage, providing implications for medical and surgical treatment. METHODS: Patients undergoing revisional breast surgery with suspicion of upper extremity lymphedema between 12/2014 and 3/2020 were offered lymphangiography. The breast and lateral/anterior trunks were visualized and blindly evaluated for axillary and inguinal lymphatic flow. A linear-weighted Cohen's kappa statistic was calculated comparing alternative drainage evaluation. Binomial regression was used to compute relative risks (RRs). Significance was assessed at alpha = 0.05. RESULTS: Eighty-six sides (46 patients) were included. Twelve sides underwent no treatment and were considered controls. Eighty-eight percent of the noncontrols had alternative lymphatic flow to the ipsilateral axillae (64%), ipsilateral groins (57%), contralateral axillae (20.3%), and contralateral groins (9.3%). Cohen's kappa for alternative drainage was 0.631 ± 0.043. Ipsilateral axillary and contralateral inguinal drainage were associated with reduced risk of developing truncal lymphedema [RR 0.78, confidence interval (CI) 0.63-0.97, P = 0.04; RR 0.32, CI 0.13-0.79, P = 0.01, respectively]. Radiation therapy increased risk of truncal and upper extremity lymphedema (RR 3.69, CI 0.96-14.15, P = 0.02; RR 1.92, CI 1.09-3.39, P = 0.03, respectively). Contralateral axillary drainage and axillary lymph node dissection were associated with increased risk of upper extremity lymphedema (RR 4.25, CI 1.09-16.61, P = 0.01; RR 2.83, CI 1.23-6.52, P = 0.01, respectively). CONCLUSIONS: Building upon previous work, this study shows risk and protective factors for the development of truncal and upper extremity lymphedema. Most prevalent alternative channels drain to the ipsilateral axilla and groin. Ipsilateral axillary and contralateral inguinal drainage were associated with reduced risk of truncal lymphedema. Patients with radiation, axillary dissection, and contralateral axillary drainage were associated with increased risk of upper extremity lymphedema. These findings have important clinical implications for postoperative manual lymphatic drainage and for determining eligibility for lymphovenous bypass surgery.

Bilateral axillary web syndrome in a patient with primary lymphoedema of upper limbs and non-Hodgkin lymphoma.

Primary lymphoedema, axillary web syndrome (AWS) and yellow nail syndrome may be related. Mr B is a 66-year-old gentleman with genital lymphoedema and lymphoedema of all four extremities. In 2023, he was diagnosed with non-Hodgkin lymphoma and also underwent cardiac surgery. In November 2023, he completed an inpatient rehabilitation at the Földi clinic in Germany, where he received intensive treatment for his lymphoedema and was also diagnosed with bilateral AWS. The presence of AWS in a patient with primary lymphoedema and no history of axillary surgery is unique. Although AWS typically presents after axillary surgery, this case highlights that it can also occur in patients without lymph node surgery. While the precise cause of this presentation of AWS is not known, it may be connected to yellow nail syndrome or potentially the recent chemotherapy treatment. This article will describe the clinical case, highlighting the need for further research on AWS present in primary lymphoedema.

[Rare nerve compression neuropathies] / Seltene Nervenkompressionssyndrome.

UNCOMMON NERVE COMPRESSION SYNDROMES: In regard to the complex anatomical relationship of peripheral nerves and muscles, tendons, fasciae as well as their long course within those anatomical structures and additional close contact to bony structures, they are prone to suffer from local compression syndromes. Hence creating a vast majority of entrapment syndromes - well described in literature for almost every single nerve. The purpose of this article is to give an overview of symptoms, signs, diagnostic studies and treatment options, addressing especially the less known syndromes. Compression syndromes of the upper arm and shoulder region include the suprascapular nerve syndrome the compression of the axillary nerve within the spatium quadrilaterale and the compression of the long thoracic nerve at the chest wall. The upper extremity offers a variety of infrequent entrapment syndromes, as the pronator teres syndrome and anterior interosseus syndrome, both resulting from pressure to the median nerve in the forearm. Compression neuropathy in the course of the radial nerve in the distal upper extremity is also known as supinator syndrome. Guyon's canal syndrome is the ulnar side equivalent to the well-known carpal tunnel syndrome. In the case of a Cheiralgia paresthetica, a compression of a sensory branch of the superficial radial nerve can be seen. In the lower extremities, a variety of nerves especially in the groin and thigh area can be compressed as they pass through the narrow spaces between the abdominal muscles or underneath the inguinal ligament. Compression of the lateral femoral cutaneous nerve is the most common syndrome. Compression syndromes of the femoral and obturator nerves are most often iatrogenic. Pain around the knee, especially the lateral part and following orthopedic procedures of the knee, can arise from a compression or a lesion of a small infrapatellar branch of the saphenous nerve. Another probably underdiagnosed syndrome is piriformis syndrome, resulting from an entrapment of the sciatic nerve as it passes through certain muscular structures. In the distal lower extremity, the peroneal and tibial nerves can be compressed at multple sites, clinically known as peroneal nerve paralysis resulting from nerve compression around the fibular head, the anterior and posterior tarsal tunnel syndrome, and Morton's metatarsalgia.

Malignant giant cell tumour of distal ulna.

Giant cell tumour (GCT) accounts for 5% of all primary bone tumours. GCT in the distal third of ulna is quite rare. We present a case of recurrent GCT in distal third of ulna with malignant features involving tenosynovium. The case was treated by wide resection of tumour and on follow up, patient recovered well with no evidence of further recurrence. Considering the features, according to the literature reviewed, is the first case of its type.

[Preventive axillary lymphovenous anastomoses simultaneously with lymph node dissection in the treatment of breast cancer for prevention of lymphedema of the upper limb (LYMPHA technique)]. / Preventivnoe nalozhenie limfovenoznykh anastomozov v podmyshechnoi oblasti odnomomentno s limfaticheskoi dissektsiei pri lechenii raka molochnoi zhelezy dlya profilaktiki limfedemy verkhnei konechnosti (metodika LYMPHA).

OBJECTIVE: To analyze the effect the LYMPHA technique on the incidence of upper limb lymphedema in patients with breast cancer after complete axillary lymph node dissection. MATERIAL AND METHODS: There were 89 patients with breast cancer and signs of metastatic lesion of axillary lymph nodes who underwent complete axillary lymph dissection. In group 1 (41 patients), the LYMPHA technique was used simultaneously with lymph node dissection; in group 2 (48 patients) - lymph node dissection alone. RESULTS: The follow-up period was 1 year. The LYMPHA technique prolonged surgery and decreased duration of postoperative lymphorrhea. The incidence of upper limb lymphedema was 9.8% and 22.9%, respectively. CONCLUSION: The LYMPHA technique was effective for prevention of upper limb lymphedema after complete axillary lymph node dissection in the treatment of breast cancer.

Durability of intercalary endoprosthesis for humeral reconstruction.

INTRODUCTION: The humerus is a common site of metastases and primary tumors. For some patients with a segmental defect and/or diaphyseal cortical destruction a cemented intercalary device may provide a more reliable construct, however data on their use is limited. METHODS: We reviewed 43 (28 male and 15 female) patients treated with an intercalary humeral spacer at a single tertiary referral center between 1989 and 2022. Humeral lesions were most commonly secondary to metastatic disease (n = 29, 68%), with 25 (58%) patients presenting with a pathologic fracture. Mean age and body mass index were 66 years and 27.9 kg/m2 . First generation taper joint device were used in 22 patients and second-generation lap device in 21 patients. RESULTS: Following reconstruction the 2-year overall survival was 30%. Mechanical complications occurred in 11 patients, most commonly aseptic loosening (n = 6, 14%). With death as a competing risk, the cumulative incidence of mechanical failure was 28% at 2-years postoperative. Following the procedure, mean Musculoskeletal Tumor Society scores was 70% and mean shoulder elevation was 87°. CONCLUSION: Reconstruction of the humeral diaphysis with an intercalary endoprosthesis provides restoration of function of the upper extremity, however, is associated with one in four patients having mechanical failure.

Risk factors for local recurrence of upper extremity desmoid tumors.

INTRODUCTION: Upper extremity (UE) desmoid tumors are locally aggressive neoplasms with high recurrence rates. Our study sought to analyze the demographics and treatment strategies of UE desmoid tumors and identify risk factors for recurrence. MATERIALS AND METHODS: A retrospective review of 52 patients with histologically confirmed UE desmoid tumors treated at our institution between 1990 and 2015 was conducted. Survival was assessed using the Kaplan-Meier method and the Cox proportional hazards model was used for risk factor analysis. RESULTS: For the entire cohort, median age was 40 (29-47) years, 75% were female, and 48% had local recurrence. The median tumor size was 45 (15-111) cm3 on imaging. Twenty-two patients had a previous resection. The most common treatments were surgery alone (50%) and surgery with adjuvant radiotherapy (21%). Tumor size ≥5 cm and tumor volume ≥40 cm3 on imaging were associated with increased recurrence (p = 0.006 and p = 0.005, respectively). Age and sex were not associated with local recurrence. Patients with a tumor size ≥5 cm were 2.6 times more likely to present with recurrence. At the 10-year mark, a lower local recurrence-free survival was seen in patients with tumors ≥5 cm (72.2% vs. 36.3%, p = 0.042) or ≥40 cm3 (67.2% vs. 32.7%, p = 0.034). CONCLUSION: In our study, only tumor dimensions appeared to modify recurrence risk.

Nodular-Type Lichen Myxedematosus: A Rare Variant With Exclusive Involvement of the Hands-Report of Two Cases With a Comprehensive Review of the Literature.

ABSTRACT: Lichen myxedematosus (LM) is an uncommon cutaneous mucinosis characterized by the deposition of mucin and fibroblast proliferation in the dermis. This condition can be classified into 2 forms: a diffuse/generalized LM, also known as scleromyxedema, associated with monoclonal gammopathy and systemic implications, and a localized form, primarily affecting the skin. Within the localized form, nodular-type LM is a rare variant presenting as firm, skin-colored to pinkish mucinous nodules. In this article, we report 2 new cases of nodular-type LM with exclusive involvement of the hands and provide a comprehensive review of the diagnosis, histopathological aspects, and therapeutic considerations of this rare condition.

Pediatric parosteal osteosarcoma of the distal radius causing extensive erosive mass effect of the adjacent ulna: a case report.

INTRODUCTION: Parosteal osteosarcomas are low-grade bony malignancies that are treated primarily with surgical resection and reconstruction. This report discusses a unique case of a pediatric patient who presented with a parosteal osteosarcoma of the distal radius causing extensive erosive mass effect and growth disturbance of the adjacent ulna. Likely due to their slow-growing nonaggressive nature, parosteal osteosarcomas have not been previously described to abut adjacent bony structures through direct contact. The patient presented in a significantly delayed manner due to social circumstances, inadvertently revealing this novel behavior. This report reviews this rare case and describes the current understanding of this tumor. CASE PRESENTATION: The patient is a 13-year-old male who presented with a parosteal osteosarcoma of his distal radius. He presented with a palpable wrist mass and wrist stiffness. He presented in a delayed manner with advanced local disease due to social factors. Imaging revealed an osseous radial mass that abutted the ulna and likely stunted its growth. The patient ultimately underwent complex resection and allograft reconstruction of both his distal radius and ulna. Intraoperative pathology was confirmed to have negative tumor margins. Allograft reconstruction of the radius and ulna was performed utilizing patient-specific custom cutting guides. At the 6-month postoperative visit, the patient had no recurrence of the mass, minimal pain, and had almost regained range of motion of the extremities. Clinical radiographs at the 6-month postoperative visit demonstrated allograft incorporation. CONCLUSIONS: A previously unreported case of pediatric parosteal osteosarcoma of the distal radius with erosion of the adjacent ulna through direct contact is presented. The challenges in and the importance of arriving at a definitive diagnosis in a timely manner for the proper treatment of this malignancy are emphasized.

Plexiform neurofibroma (Hamartoma) of the median nerve: A two-case report.

Plexiform neurofibroma is a benign peripheral nerve-sheath tumor, rarely involving major nerves of the extremities. In the literature, there are no clear treatment strategies for plexiform neurofibroma of major peripheral nerves. Our experience encountered two patients with plexiform neurofibroma of the median nerve, presenting with a palmar mass and symptoms of carpal tunnel compression. Preoperatively, plexiform neurofibroma was diagnosed on MRI and clinical examination. Both patients also experienced significant neurological deterioration, with finger numbness and increased nerve/tumor size. Potential malignant transformation was also considered. For these reasons, resection of the involved area of the nerve and repair were indicated. In both patients, intraoperative pathological diagnosis was plexiform neurofibroma. The 45-year-old male patient refused further surgery after carpal tunnel release, which was performed under axillary block. One year postoperatively, nerve compression symptoms decreased moderately. In the other patient, a 7-year-old boy, a significantly enlarged area of the median nerve was resected, and neurorrhaphy was performed. One year postoperatively, median nerve motor-sensory functions recovered completely. Four years postoperatively, no enlargement of the residual tumor was observed.

Spindle-cell myoepithelioma: A rare case in a finger of a 15-year-old boy.

Cutaneous myoepithelioma is a rare neoplasm of the skin that has become more widely recognized in recent years despite significant diagnostic pitfalls. It is a benign neoplasm with a high recurrence rate if not excised radically, and must be distinguished from its malignant counterpart. Few cases have been described so far and, to our knowledge, no cases in the finger of a child exist in the literature. We report the case of a 15 year-old boy affected by a rare form of locally aggressive spindle-cell myoepithelioma, and suggest a new multidisciplinary approach combining surgical excision and custom brachytherapy.

Upper Limb Morbidity in Newly Diagnosed Individuals After Unilateral Surgery for Breast Cancer: Baseline Results from the AMBER Cohort Study.

PURPOSE: We aimed to examine potential associations between post-surgical upper limb morbidity and demographic, medical, surgical, and health-related fitness variables in newly diagnosed individuals with breast cancer. METHODS: Participants were recruited between 2012 and 2019. Objective measures of health-related fitness, body composition, shoulder range of motion, axillary web syndrome, and lymphedema were performed within 3 months of breast cancer surgery, and prior to or at the start of adjuvant cancer treatment. RESULTS: Upper limb morbidity was identified in 54% of participants and was associated with poorer upper limb function and higher pain. Multivariable logistic regression analysis identified mastectomy versus breast-conserving surgery (odds ratio [OR] 3.51, 95% confidence interval [CI] 2.65-4.65), axillary lymph node dissection versus sentinel lymph node dissection (OR 2.67, 95% CI 1.73-4.10), earlier versus later time from surgery (OR 1.58, 95% CI 1.15-2.18), and younger versus older age (OR 1.01, 95% CI 1.00-1.03) as significantly associated with a higher odds of upper limb morbidity, while mastectomy (OR 1.57, 95% CI 1.10-2.25), axillary lymph node dissection (OR 2.20, 95% CI 1.34-3.60), lower muscular endurance (OR 1.10, 95% CI 1.01-1.16) and higher percentage body fat (OR 1.04, 95% CI 1.00-1.07) were significantly associated with higher odds of moderate or greater morbidity severity. CONCLUSIONS: Upper limb morbidity is common in individuals after breast cancer surgery prior to adjuvant cancer treatment. Health-related fitness variables were associated with severity of upper limb morbidity. Findings may facilitate prospective surveillance of individuals at higher risk of developing upper limb morbidity.

Spontaneous radial nerve palsy with hourglass-like constriction.

Hourglass-like constriction (HLC) is an uncommon spontaneous mononeuropathy that is typically characterised by a sudden onset of pain followed by palsy, affecting branches of the radial (posterior interosseous nerve) and median nerves (anterior interosseous nerve). HLC of the radial nerve (RN) is rare, with only a few reported cases. Here, we report a case of a man who presented with acute wrist and finger drop due to the HLC of the RN. Surgery was recommended 5 months after clinical observation, when the lesion was resected and primarily repaired, resulting in satisfactory recovery. There is still much that remains unknown about HLC, especially for RN. The current understanding points out an inflammatory disease that should be treated conservatively for 3-7 months. The surgical technique depends mostly on the severity and extent of constriction; however, considering only RN constrictions, primary repair by neurorrhaphy or nerve grafts resulted in better functional outcomes.

Fluid Distribution Patterns in Early-Stage Upper Extremity Lymphedema.

INTRODUCTION: Magnetic resonance imaging (MRI) stage 1 (early stage) upper extremity lymphedema is characterized by fluid infiltration in the subcutaneous tissues that does not exceed 50% of the extremity circumference at any level. The spatial fluid distribution in these cases has not been detailed and may be important to help determine the presence and location of compensatory lymphatic channels. The aim of this study is to determine whether there was a pattern of distribution of fluid infiltration in patients with early-stage lymphedema that could correspond to known lymphatic pathways in the upper extremity. METHODS: A retrospective review identified all patients with MRI stage 1 upper extremity lymphedema who were evaluated at a single lymphatic center. Using a standardized scoring system, a radiologist graded the severity of fluid infiltration at 18 anatomical locations. A cumulative spatial histogram was then created to map out regions where fluid accumulation occurred most and least frequently. RESULTS: Eleven patients with MRI stage 1 upper extremity lymphedema were identified between January 2017 and January 2022. The mean age was 58 years and the mean BMI was 30 m/kg2. One patient had primary lymphedema and the remaining 10 had secondary lymphedema. The forearm was affected in nine cases, and fluid infiltration was predominantly concentrated along the ulnar aspect, followed by the volar aspect, while the radial aspect was completely spared. Within the upper arm, fluid was primarily concentrated distally and posteriorly, and occasionally medially. CONCLUSIONS: In patients with early-stage lymphedema, fluid infiltration is concentrated along the ulnar forearm and the posterior distal upper arm, which aligns with the tricipital lymphatic pathway. There is also sparing of fluid accumulation along the radial forearm in these patients, suggesting a more robust lymphatic drainage along this region, possibly due to a connection to the lateral upper arm pathway.

Reliable Location of Upper Extremity Lymphatic Channels for Use in Immediate Lymphatic Reconstruction.

BACKGROUND: Breast cancer-related lymphedema (BCRL) is a limiting sequelae of breast cancer treatment that may negatively impact 30% to 50% of high-risk breast cancer survivors. Risk factors for development of BCRL include axillary lymph node dissection (ALND), and recently, axillary reverse lymphatic mapping and immediate lymphovenous reconstruction (ILR) at time of ALND have been implemented to prevent BCRL. Reliable anatomy of neighboring venules has been commented on in the literature; however, little information exists about anatomical location of local lymphatic channels amenable for bypass. METHODS: After institutional review board approval, patients who underwent ALND with axillary reverse lymphatic mapping and ILR at a tertiary cancer center from November 2021 to August 2022 were applicable for this study. The location and number of lymphatic channels used for ILR were identified and measured intraoperatively with the arm abducted to 90 degrees and soft tissue under no tension. Four measurements were taken to localize each lymphatic and were based on relationship with reliable anatomic landmarks including 4th rib, anterior axillary line, and lower border of the pectoralis major muscle. Demographics, oncologic treatments, intraoperative factors, and outcomes were prospectively maintained. RESULTS: Twenty-seven patients met inclusion for this study by August 2022 with a total of 86 lymphatic channels identified. Patients were on average 50 ± 12 years old with a body mass index of 30 ± 6 and had an average of 1 vein and 3 identifiable lymphatic channels amenable to bypass. Seventy percent of lymphatic channels were found in a cluster of 2 or more channels. The average horizontal location was 4.5 ± 1.4 cm lateral to the 4th rib. The average vertical location was 1.3 ± 0.9 cm from the superior border of the 4th rib. CONCLUSIONS: These data comment upon intraoperatively identified and consistent location of upper extremity lymphatic channels used for ILR. These lymphatic channels are often found in clusters with 2 or more lymphatic channels at the same location. Such insight may aid in easier intraoperative identification of amenable vessels for the unexperienced surgeon, decrease in intraoperative time, and higher success of ILR.

Reporting of scar outcomes in the hand and wrist; a state-of-the-art literature review.

OBJECTIVES: The aim of this literature review was to synthesise and report current practice in evaluation and reporting of scar outcomes in hand and wrist clinical research. METHODS: A systematic search from inception to 2022 was conducted using three electronic databases. English language randomized controlled trials and observational cohort studies reporting standardised scar outcome measures and/or scar symptoms, appearance, impairment, function, or mental health outcomes in patients with hand and wrist scars were included. Two independent reviewers determined study eligibility and performed data extraction of a priori identified scar outcome domains. Data analysis included descriptive statistics and identification of discordance in taxonomy. RESULTS: Fifty-nine studies were included. Elective surgery cohorts were the most frequently included clinical population (n = 28; 47%) followed by burns (n = 16; 27%). Six different standardised scar outcome measures were reported by 25% of studies however only 7% of studies utilised a patient-reported measure. Scar symptoms were the most frequently reported outcome domain (81%); but taxonomy was incongruous, constructs lacked working definitions required for generalisability and outcome measurement was variable and unreported. Nineteen different measures of scar appearance and structure were reported by 30 (51%) of studies however only nine (23%) were patient-reported. Seven different hand function PROMs were reported by 25 (43%) studies. Person-centred domains including scar acceptability (12%), mental health impact (5%), and social participation (4%) were rarely reported. CONCLUSIONS: This review highlights that evaluation and reporting of hand and wrist scar outcomes is not standardised, assessment methods and measures are under-reported and there is discordance in taxonomy. Evaluation is not person-centred, rather it is dependent on clinician assessment. Domains including scar acceptability, mental health, and social participation are rarely addressed. A stakeholder consensus derived hand and wrist scar core outcome measurement set will promote standardisation and underpin improvements in clinical research quality, transparency, and rigour.

The flubbed body: Pathological body size representation in personal neglect.

Personal Neglect (PN) is a disorder in which patients fail to attend or explore the contralateral side of their body. An increasing number of studies have considered PN as a form of body representation disorder frequently observed following damage to parietal areas. The extent and the direction of the body misrepresentation is still unclear with recent studies suggesting a general reduction of contralesional hand size. However, little is known about the specificity of this representation and whether the misrepresentation also generalises to other body parts. We explored the features of the representation of the hands and face in a group of 9 right brain damaged patients with (PN+) and without PN (PN-), when compared to a healthy control group. For this, we used a body size estimation task with pictures, in which patients were required to choose the one that most closely matched the perceived size of their body part. We found that PN + patients showed a labile body representation for both hands and face, having a larger distorted representational range. Interestingly, in comparison with PN + patients and healthy controls, PN- patients also showed misrepresentation of the left contralesional hand which could be related to impaired motor performance of their upper limb. Our findings are discussed within a theoretical framework suggesting a reliance on multisensory integration (body representation, ownership, and motor influences) for an ordered representation of the size of the body.

[Plexiform fibrohistiocytic tumor]. / Plexiformer fibrohistiozytärer Tumor.

Plexiform fibrohistiocytic tumors are rare, low-to-moderate malignant soft tissue tumors that occur primarily in children and adolescents and are located on the upper extremity. The diagnosis must be made histologically. We report on a young woman who presented a growing, painless lesion on the cubital fossa. Histopathology as well as the standard of treatment are discussed.