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Primary cutaneous epidermotropic alveolar rhabdomyosarcoma with t(2;13) in an elderly woman: case report and review of the literature.

We report a case of a primary cutaneous alveolar rhabdomyosarcoma presenting on the lower limb of a 60-year old woman. The tumor was characterized by aggregates of round blue cells in an alveolar growth pattern in the dermis and subcutis, with the additional unique finding of epidermotropism. By immunohistochemistry tumor cells were positive for vimentin, muscle-specific actin, desmin, myogenin, and Myo-D1 with focal positivity for CD56, neuron-specific enolase, and S-100 protein. Staining for pan-keratin, HMB-45, melan-A, epithelial membrane antigen, chromogranin, CD99, leukocyte common antigen, and alpha-smooth muscle actin was negative. Interphase fluorescence in situ hybridization analysis from paraffin-embedded tumor demonstrated the presence of the translocation (2;13)(q35;q14) confirming the diagnosis. Further investigations revealed no tumor in the underlying deep soft tissues, and there was no evidence of metastasis in other organs. A local recurrence associated with a metastasis to a regional lymph node on the right groin was treated with an above-knee amputation and local radiotherapy to the groin area. The patient subsequently developed cutaneous metastases in the amputation stump and died 2 years after initial presentation. This case indicates that rhabdomyosarcoma may rarely present in the skin in adults and should be included in the differential diagnosis of primary cutaneous small round blue cell tumors not only in children but also in this age group.

Chromosomes, Human, Pair 13↗

Cross-foot island instep flap: a new use of instep skin flap for management of persistent wounds after complex plantar foot reconstruction.

When presented with an extensive soft-tissue defect involving the sole of the foot, reconstruction with free muscle flaps covered by a split-thickness skin graft is the proposed method of treatment. However, persistent graft breakdown and a chronic wound of the weight-bearing flap is a challenging problem during the late postoperative period, as experienced by the authors in their patients with high-energy-induced lower extremity injuries. The authors used the instep flap as an island cross-foot flap to manage persistent graft breakdown that involved skin-grafted muscle flaps transferred previously to the heel in 3 patients and to treat a chronic wound involving an amputation stump in 1 patient. The vascular pathology of the injured extremities indicated a cross-leg procedure instead of a free flap transfer. Pedicles were wrapped with split-thickness skin grafts and flaps were harvested superficial to the plantar fascia. Pedicles were divided during postoperative week 3, and no complications related to the operation or to immobilization have been encountered during the postoperative follow-up. During the 1-year follow-up, durable coverage, free from development of open wounds, has been achieved, and patients have expressed their satisfaction. In the case of complicated, high-velocity foot injuries, the authors suggest that this procedure be kept in mind as an alternative treatment option because it has some advantages over conventional cross-leg procedures.

Adult↗

Review of the effectiveness of capsaicin for painful cutaneous disorders and neural dysfunction.

BACKGROUND: Topical capsaicin is known to be a safe and effective pain management adjunct for rheumatoid arthritis, osteoarthritis, neuralgias, and diabetic neuropathy. However, studies and case reports in the literature have indicated that other conditions may also benefit from capsaicin: painful or itching cutaneous disorders from operations, injuries, or tumors; neural dysfunction; or inflammation of the airways and urinary tract. METHODS: To determine the effectiveness of capsaicin for painful cutaneous disorders and neural dysfunction, the authors analyzed data from 33 reports (MEDLINE search of 1966-96) on the efficacy of capsaicin. Outcome measures consisted of the response rate and degree of pain relief. Results from placebo-controlled trials were pooled when possible; effect of treatment was estimated by the method of DerSimonian and Laird. RESULTS: Pain relief for postmastectomy syndrome and cluster headache was greater with capsaicin than with placebo; also, psoriasis and pruritus responded better to capsaicin. Uncontrolled studies and case reports have indicated that pain or dysfunction was less at the end of capsaicin therapy for neck pain, loin pain/hematuria syndrome, oral mucositis, rhinopathy, reflex sympathetic dystrophy syndrome, detrusor hyperreflexia, and cutaneous pain due to tumor of the skin. CONCLUSIONS: Capsaicin is effective for psoriasis, pruritus, and cluster headache; it is often helpful for the itching and pain of postmastectomy pain syndrome, oral mucositis, cutaneous allergy, loin pain/hematuria syndrome, neck pain, amputation stump pain, and skin tumor; and it may be beneficial for neural dysfunction (detrusor hyperreflexia, reflex sympathetic dystrophy, and rhinopathy). A universal problem for many of the studies analyzed was the absence of a "burning placebo" such as camphor.

Capsaicin↗

Amputation salvage with microvascular free flap from the amputated extremity.

Microvascular surgery has allowed creative primary reconstruction using undamaged parts of amputated limbs as donor sites. We present a case of lower extremity below-knee amputation salvage with a free flap of heel, calcaneus, and soft tissues from the amputated lower leg, ankle, and foot supplied by the posterior tibial neurovascular bundle.

Adult↗

Tissue expansion: a method to preserve bone length and joints following traumatic amputations of the leg--a follow-up of five legs amputated at different levels.

Tissue expansion of adjacent intact skin and subcutaneous tissue has in five legs provided high-quality soft-tissue coverage of the distal end in legs amputated at different levels without further shortening of the bone. The sensitivity of slowly expanded flaps is temporarily affected but eventually seems to return to normal. Expanded flaps have endured both sitting and the use of prostheses for 4 to 5 years. In one case the technique made it possible to save the knee joint. Besides being a functional reconstruction, the appearance of the reconstructed parts much improved. A technique of overexpansion and double flap coverage of the bone utilizing deepithelialization is described.

Adolescent↗

Iatrogenic fetal injury.

BACKGROUND: Iatrogenic fetal injury during cesarean delivery is a serious but underreported complication. CASE: A distal iatrogenic amputation of a digit occurred during a cesarean delivery. CONCLUSION: Obstetricians should be aware of this potential complication.

Adult↗

Verrucous hyperplasia of the great toe: a case and a review of the literature.

BACKGROUND: Verrucous hyperplasia is a hyperplastic disease that manifests as coalescent warty papules at stump amputation sites. Often overlooked, it can be confused with verrucous carcinoma. As such, it is of concern for dermatologic surgeons. OBJECTIVE: To present a patient with verrucous hyperplasia and review its literature. METHODS: We report a 62-year-old diabetic patient with a verrucous nodule that arose at the amputation site of her first three right toes. Despite repeated surgical removals and skin graftings, the verrucous nodule redeveloped. A biopsy revealed digitate epidermal hyperplasia with dilated tortuous capillaries in thin dermal papilla without invasive features, mitotic figures, or acanthotic downgrowth. Polymerase chain reaction did not detect human papilloma virus. RESULTS: Verrucous hyperplasia secondary to amputation was diagnosed. CONCLUSION: Verrucous hyperplasia occurs should not be confused with verrucous carcinoma or warts. Moreover, because it recurs after removal, surgery is not indicated; rather, compression therapy is indicated.

Amputation, Surgical↗

Flavobacterium odoratum: a species resistant to a wide range of antimicrobial agents.

During the period 1966-77, 24 strains of Flavobacterium odoratum were identified from among strains of Gram-negative, non-fermentative bacteria submitted to the National Collection of Type Cultures for computer-assisted identification. The F. odoratum strains showed resistance to therapeutic levels of gentamicin, tobramycin, amikacin, and carbenicillin as well as to several other antimicrobial agents generally useful in the treatment of infections caused by Gram-negative, non-fermentative bacteria. Two strains isolated from amputation stumps and another three strains isolated in significant numbers from urine specimens were possibly opportunist pathogens. The biochemical characteristics of the 24 strains, the proposed neotype strain of F. odoratum, and three strains representative of a group, referred to at the Center for Disease Control, Atlanta as group M-4f, were compared with those of biochemically similar species which may be isolated from clinical material.

Anti-Bacterial Agents↗

Psychophysical correlates of phantom limb experience.

Phantom limb phenomena were correlated with psychophysiological measures of peripheral sympathetic nervous system activity measured at the amputation stump and contralateral limb. Amputees were assigned to one of three groups depending on whether they reported phantom limb pain, non-painful phantom limb sensations, or no phantom limb at all. Skin conductance and skin temperature were recorded continuously during two 30 minute sessions while subjects continuously monitored and rated the intensity of any phantom limb sensation or pain they experienced. The results from both sessions showed that mean skin temperature was significantly lower at the stump than the contralateral limb in the groups with phantom limb pain and non-painful phantom limb sensations, but not among subjects with no phantom limb at all. In addition, stump skin conductance responses correlated significantly with the intensity of non-painful phantom limb paresthesiae but not other qualities of sensation or pain. Between-limb measures of pressure sensitivity were not significantly different in any group. The results suggest that the presence of a phantom limb, whether painful or painless, is related to the sympathetic-efferent outflow of cutaneous vasoconstrictor fibres in the stump and stump neuromas. The hypothesis of a sympathetic-efferent somatic-afferent mechanism involving both sudomotor and vasoconstrictor fibres is proposed to explain the relationship between stump skin conductance responses and non-painful phantom limb paresthesiae. It is suggested that increases in the intensity of phantom limb paresthesiae follow bursts of sympathetic activity due to neurotransmitter release onto apposing sprouts of large diameter primary afferents located in stump neuromas, and decreases correspond to periods of relative sympathetic inactivity. The results of the study agree with recent suggestions that phantom limb pain is not a unitary syndrome, but a symptom class with each class subserved by different aetiological mechanisms.

Adult↗

From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation.

Numerous neurogenic tumors can affect the musculoskeletal system, including traumatic neuroma, Morton neuroma, neural fibrolipoma, nerve sheath ganglion, neurilemoma, neurofibroma, and malignant peripheral nerve sheath tumors (PNSTs). The diagnosis of neurogenic tumors can be suggested from their imaging appearances, including lesion shape and intrinsic imaging characteristics. It is also important to establish lesion location along a typical nerve distribution (eg, plantar digital nerve in Morton neuroma, median nerve in neural fibrolipoma, large nerve trunk in benign and malignant PNSTs). Traumatic and Morton neuromas are commonly related to an amputation stump or are located in the intermetatarsal space, respectively. Neural fibrolipomas show fat interspersed between nerve fascicles and are often associated with macrodactyly. Nerve sheath ganglion has a cystic appearance and commonly occurs about the knee. Radiologic characteristics of neurilemoma, neurofibroma, and malignant PNST at computed tomography (CT), ultrasonography, and magnetic resonance imaging include fusiform shape, identification of entering and exiting nerve, low attenuation at CT, target sign, fascicular sign, split-fat sign, and associated muscle atrophy. Although differentiation of neurilemoma from neurofibroma and of benign from malignant PNST is problematic, recognition of the radiologic appearances of neurogenic tumors often allows prospective diagnosis and improves clinical management of patients.

Adult↗

Hypnotic imagery as a treatment for phantom limb pain: two case reports and a review.

OBJECTIVE: To provide a theoretical background, to review existing literature and to present new case material relevant to the treatment of phantom limb pain using hypnotic imagery. METHOD: This paper presents two new case reports involving the use of hypnotic imagery procedures in the alleviation of phantom limb pain and reviews 10 previous clinical studies which have involved a similar approach. The earlier studies were identified by electronic and manual searches of the relevant literature. RESULTS: Two main treatment strategies can be identified: (1) ipsative/imagery-based approaches and (2) movement/imagery-based approaches. A common finding is the need to treat the phantom limb as a 'real' body part, to accept its existence as a valid mental representation and to avoid treating the amputation stump as the sole source of the phantom pain sensations. CONCLUSION: Hypnotic procedures appear to be a useful adjunct to established strategies for the treatment of phantom limb pain and would repay further, more systematic, investigation. Suggestions are provided as to the factors which should be considered for a more systematic research programme.

Aged↗