Gonadal dysgenesis patients with Y chromosomal material are subject to increased risk for germ cell tumors. We report a case of an adolescent female presenting with Turner-like syndrome with primary amenorrhea and Tanner stage 1 breast development. Karyotype showed one X chromosome and a minute pericentromeric fragment of Y chromosome without any functional Y genes in all the cells, unlike a mosaic pattern, represented as 46,X,der(Y)del(Y)(p11.2)del(q11.2). Laparoscopic bilateral gonadectomy was performed due to presence of Y chromosome material and histopathology confirmed gonadoblastoma with a focus of dysgerminoma of the right ovary. A robotic-assisted surgical staging for dysgerminoma was performed which was confirmed to be negative for malignancy. This points at the putative genes for gonadoblastoma to be present around the centromere of the Y chromosome.
Solitary fibrous tumours (SFTs) are rare tumours arising from mesenchymal tissues. Despite of their more frequent occurrence in the pleura, SFT can present anywhere in the body. Only a few cases have been described arising from the mesentery. Most tumours have a benign nature; however, up to 20% of them can spread, most commonly to liver, lung and bone. Surgical excision including all surrounding tissues remains the treatment of choice; however, there is no consensus regarding the need for adjuvant therapies. We present a 79-year-old man with abdominal pain who was found to have a SFT in the mesentery of the sigmoid colon, treated with en bloc resection. A multidisciplinary team including surgeons, medical and radiation oncologists is recommended in the care of these patients.
Extramedullary plasmacytoma is a type of plasma cell dyscrasia that can present
as solitary tumor or secondary to multiple myeloma. We experienced a case of
intramuscular plasmacytoma in the left thigh muscles of a patient secondary to
multiple myeloma. A 73-year-old male with relapsed multiple myeloma and
bilateral hip arthroplasty complained of lxeft lower limb weakness and hip pain
3 months after relapse. He underwent contrast-enhanced magnetic resonance
imaging of lumbar spine and hip which was inconclusive. Subsequently, patient
had multiple admissions for progressive lower limb weakness. His clinical course
was complicated by a biopsy-proven plasmacytoma of the neck. He received
localized radiation therapy to the neck in addition to a change in multiple
myeloma chemotherapy regimen, resulting in resolution of the neck mass but his
left lower extremity weakness continued to worsen. Repeat magnetic resonance
imaging of hip and spine revealed an intramuscular mass in left thigh which was
consistent with the diagnosis of extramedullary plasmacytoma on biopsy.
Localized radiation to the thigh accompanied with a change in chemotherapy
improved his symptoms and a significant reduction in size of plasmacytoma was
observed. When an unexplained lower limb weakness is encountered with a history
of multiple myeloma, secondary intramuscular plasmacytoma should be
considered.
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