We performed a successful penis allotransplantation on 11 December 2014. Sharing the lessons learned might help more patients in need to be treated this way. We divided the project into manageable segments that was each overseen by an expert. The ethical review and conduct paved the way for a publically acceptable and successful project. Screening for a psychological stable recipient is important. The most difficult part of the project was finding a donor penis. This was successfully negotiated with the family of a brain dead donor by creating a neo-phallus for the donor, thereby maintaining the dignity of the donor. Working with transplant coordinators that are sympathetic to aphallic men is crucial. Surgeons versed in microvascular techniques is a critical part of the team. Transplant immunologists have to adapt to treat composite tissue transplantation patients.
Adamantinoma-like Ewing sarcoma is a rare variant of Ewing sarcoma with histologic and immunohistochemical evidence of squamous differentiation. This variant most commonly occurs in the head and neck region with a few cases reported in the long bones of the limbs. It may be associated with poorer clinical outcome and could pose a diagnostic challenge, particularly if it occurs in older patients or as a metastatic lesion. We present a case of Ewing sarcoma in the metatarsal of an 11-year-old boy that manifested adamantinoma-like morphology after neoadjuvant chemotherapy. Chemotherapy has been reported to induce neuronal maturation and rhabdoid morphology in cases of Ewing sarcoma, but no reports of treatment-induced squamous differentiation with P40/P63 expression have been demonstrated. This is also the first documented case treated with a pedicled osteocutaneous fibular transfer in a metatarsal malignancy, which is usually treated by either ray or below-knee amputation.
Hidradenitis suppurativa (HS, from the Greek hidros = sweat, and aden = glands) is a chronic inflammatory skin condition that is also known as acne inversa (AI). [1] HS/AI is a chronic follicular occlusive disease involving the follicular portion of folliculopilosebaceous units (FPSUs). [1] The prevalence of HS/AI has varied, ranging from less than 1% to 4%. [2] The onset of symptoms typically occurs between puberty and age 40, most commonly in the second or third decade of life. [2] The incidence of HS/AI is twice as high among women than men. [2] Genetic susceptibility, mechanical stresses on the skin, obesity, smoking, diet and hormonal imbalances are repeatedly cited as factors that may be associated with the development or exacerbation of HS/AI. [3] The primary sites of involvement for HS/AI are the intertriginous skin areas of the axillary (61%), groin (48%), perianal (30-50%), perineal (22%) and inframammary regions (13%), though it can occur in any skin area that contains FPSUs. [2] The clinical manifestations vary, ranging from recurrent inflamed nodules and abscesses to draining sinus tracts and bands of severe scar formation. [3] The associated pain, malodour, drainage and disfigurement that accompany HS/AI contribute to its profound psychosocial impact on many patients. [3] Case report An 18-year-old female was diagnosed with bilateral axillary HS in 2014. She was a known smoker, with a body mass index of 21, and no known medical comorbidities. Clinical findings She presented in 2017 with advanced Hurley stage III disease, with extensive painful open wounds extending from both axillae onto the chest wall and upper arms, and threatening to erode the left brachial vessels (Fig.1). The patient also had severe bilateral shoulder adduction contractures and reduced function, with oedema of the left arm. The perineum, perianal and groin regions were not involved. Laboratory investigations Laboratory tests showed: white cell count of 27.25 × 109/L with predominant neutrophils; haemoglobin 7.8 g/dL; haematocrit 15%; platelet
Adamantinoma-like Ewing sarcoma is a rare variant of Ewing sarcoma known to have histologic and immunohistochemical evidence of squamous differentiation. This variant most commonly occurs in the head and neck region with a few cases reported in long bones of the limbs. It may be associated with poorer clinical outcome and could pose a diagnostic challenge(s) particularly if it occurs in older patients or as a metastatic lesion. We present a case of Ewing sarcoma in the metatarsal of an 11 year old boy that manifested adamantinoma-like morphology after neo-adjuvant chemotherapy. Chemotherapy has been reported to induce neuronal maturation and rhabdoid morphology in cases of Ewing sarcoma, but no reports of treatment induced squamous differentiation with P40/P63 expression have been demonstrated. This is also the first documented case to use a pedicled osteocutaneous fibular transfer in a metatarsal malignancy, which is usually treated by either ray or below knee amputation.
Adamantinoma-like Ewing sarcoma is a rare variant of Ewing sarcoma known to have histologic and immunohistochemical evidence of squamous differentiation. This variant most commonly occurs in the head and neck region with a few cases reported in long bones of the limbs. It may be associated with poorer clinical outcome and could pose a diagnostic challenge(s) particularly if it occurs in older patients or as a metastatic lesion. We present a case of Ewing sarcoma in the metatarsal of an 11 year old boy that manifested adamantinoma-like morphology after neo-adjuvant chemotherapy. Chemotherapy has been reported to induce neuronal maturation and rhabdoid morphology in cases of Ewing sarcoma, but no reports of treatment induced squamous differentiation with P40/P63 expression have been demonstrated. This is also the first documented case to use a pedicled osteocutaneous fibular transfer in a metatarsal malignancy, which is usually treated by either ray or below knee amputation.
Background: Adamantinoma-like Ewing sarcoma is a rare variant of Ewing sarcoma known to have cytokeratin expression and squamous differentiation. It occurs more commonly in the head and neck region with only a few cases reported in long bones of the limbs. It also harbours EWSR1-FLI1 gene fusion which is required for the diagnosis of Ewing sarcoma. We present a case of Ewing sarcoma that manifested Adamantinoma-like morphology only post chemotherapy. Chemotherapy has been reported to induce neuronal maturation and rhabdoid morphology in cases of Ewing sarcoma, but no reports of treatment induced squamous differentiation with P40/P63 expression have been demonstrated to date.Case presentation: An 11 year old boy presented with a one year history of an enlarging painless mass over the left first metatarsal. Initial biopsy showed Ewing sarcoma with typical morphological features and EWSR1 rearrangement confirmed on fluorescent in-situ hybridization. The patient underwent neo-adjuvant chemotherapy and a subsequent wide local excision with an ipsilateral pedicled osteocutaneous fibula transfer. Subsequent histological examination showed frank squamous differentiation in the soft tissue component with keratin pearl formation and P40/P63 expression which was not observed in the initial biopsy and is compatible with Adamantinoma-like Ewing sarcoma.Conclusion: This case describes Adamantinoma-like Ewing sarcoma with P40/P63 expression after neo-adjuvant chemotherapy treatment. This immunophenotype was not apparent on the initial biopsy. It remains uncertain as to the reason for this change. This variant carries a poorer clinical outcome compared to the more conventional variant and could pose a diagnostic challenge(s) particularly if it occurs in an older patient or as a metastatic lesion.
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