Background: Desmoplastic small round-cell tumor (DSCRT) in adults is an extremely rare (age-adjusted incidence 0.3 per million) and aggressive sarcoma with limited data for optimal management. Patients and Methods: Retrospective analysis of patients with DSCRT diagnosis (2010DSCRT diagnosis ( -2020 was performed following Institutional Review Board approval. The follow-up period was from pathological diagnosis to the last patient contact. Endpoints were type of response and duration of response. Results: In the current analysis, first-line treatment in all cases was vincristine, anthracycline, and cyclophosphamide alternating with ifosfamide and etoposide (VAC-IE) with 100% response for a mean duration of 9.8 (range=5-12) months. Patients received 1-4 subsequent lines of therapy. All patients received temozolomide with irinotecan (50% partial response, duration 8-9 months). Two patients that underwent consolidative cytoreductive surgery with hyperthermic intraperitoneal chemotherapy had a longer survival (30.6 vs. 11.2 months). Patients suffered 100% mortality with a median survival was 17.8 (range=11.2-30.6) months. Conclusion: While aggressive multimodality treatment is always warranted for DSCRT, the options are limited by the multicentric presentation, short-lived initial response and lack of established subsequent therapy portending a poor prognosis. Consolidative cytoreductive surgery following first-line therapy may improve survival.Desmoplastic small round-cell tumor (DSCRT) in adults is a rare sarcoma predominantly affecting young adults, with an 3859 This article is freely accessible online.
Mammary Paget disease (MPD) is a malignant lesion of the nipple-areolar complex and considered an intraepidermal sign of an underlying invasive or in situ carcinoma. In rare instances, mammary Paget cells can invade the dermis. Comprehensive literature review identified 33 such cases. Here, we report a case of a 48-year-old female with invasive MPD, without an associated underlying breast cancer. Only five such presentations are reported. With a presentation of right nipple-areolar excoriation for 2 years, skin punch biopsy was performed and reported as “Paget disease.” Further evaluation with bilateral mammograms failed to show any primary mass or calcifications. Bilateral breast MRI revealed focal nonmass enhancement in upper outer quadrant in both breast, the biopsy of which showed benign breast tissue. Right breast nipple areolar complex resection demonstrated MPD extensively involving the epidermis. Multiple foci of invasive ductal carcinoma are present, growing downward into the nipple dermis, the largest focus being 0.2 cm in greatest dimension. No lymphovascular invasion is identified. The mammary Paget cells are positive for Cam 5.2 and epithelial membrane antigen (EMA) and negative for keratins 7 and 20. The invasive tumor cells are strongly positive for estrogen receptor (100%) and progesterone receptor (75%) and equivocal (2+) for Her2/Neu. FISH analysis showed amplification for HER2 (HER2/CEP17 ratio: 2.75). Four right axillary sentinel lymph nodes are negative for carcinoma. Two of the five patients with invasive MPD described in the literature, and without underlying breast cancer, had a sentinel lymph node biopsy performed. Isolated tumor clusters were present in one of these two cases. In summary, we describe a rare case of invasive MPD without an underlying breast cancer. Although sentinel lymph nodes are important to assess metastasis, further cases are required to evaluate the significance and prognosis of this rare entity.
Background/Aim: Primary small cell neuroendocrine carcinoma (SCNEC) of the adrenal gland is extremely rare with limited reports in the literature. There remain no definitive treatment guidelines, largely due to the rarity of the malignancy. Case Report: We present the case of a 62-year-old Caucasian male who presented with low back pain and was found to have a large retroperitoneal mass arising from the left adrenal gland, measuring 18.3 × 12.2 centimeters (cm). Biopsy was consistent with small cell carcinoma/high grade neuroendocrine carcinoma. Staging workup including CT chest and bone scan was negative. The patient was treated with chemotherapy, radiation therapy, and surgery; complete pathological response of the left adrenal tumor was achieved. Surveillance imaging every three months continued to show no evidence of recurrent disease. Conclusion: Primary SCNEC of the adrenal gland is rare and lacks standard treatment guidelines. Our case represents a possible treatment approach that may provide better clinical outcomes, however, further investigations are necessary to help define ideal treatment guidelines.Neuroendocrine tumors (NETs) are neoplasms that develop from neural and hormonal cell types and comprise an extensive group of tumors, which most commonly occur in the lungs, small intestine, appendix, and pancreas. Less frequently, NETs arise from the thyroid, parathyroid, pituitary, and adrenal glands (1). NETs arising in the adrenal glands are typically pheochromocytomas and paragangliomas (2). However, small-cell neuroendocrine carcinoma (SCNEC) arising from the adrenal gland is an exceedingly rare disease with limited cases in the literature. Given the rarity of the disease, treatment guidelines are not well established due to the lack of large, randomized trials. We present a case of a 62-year-old Caucasian male diagnosed with a locally advanced primary SCNEC of the adrenal gland who was treated with chemoradiation followed by surgical resection with a complete pathological response. We then review the available literature to further elucidate information regarding the diagnosis, treatment, and prognosis of SCNEC of the adrenal gland.
An 80-year-old man who was previously diagnosed with Philadelphia+ B cell-acute lymphoblastic leukaemia (B-ALL) in remission post-allogeneic matched unrelated donor peripheral blood stem cell transplant. Five years later, he was found to have unilateral testicular relapse of Philadelphia+ B-ALL proven by pathology after radical orchiectomy. Bone marrow aspirate and biopsy did not show evidence of leukaemia. Patient was treated with adjuvant radiation therapy and started on dasatinib 50 mg daily. Given his age and absence of disseminated acutelymphoblastic leukaemia (ALL), no adjuvant chemotherapy was utilised. He is monitored with monthly PCR studies. At 1-year follow-up, no findings suggestive of recurrence of ALL have been identified and the patient is maintained on the dasatinib. Although isolated testicular recurrence is common among paediatric population, it is a rare event among adults as it is considered an immunological sanctuary for cancer cells.
IntroductionCardiac amyloidosis is a rare entity with a grave prognosis. Due to the low index of suspicion secondary to non-specific symptoms, it is often diagnosed at an advanced stage with multi-organ involvement. MethodsWe report a case of systemic AL amyloidosis with predominant cardiac and renal involvement associated with multiple myeloma.
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