γδ T-cells represent a minor T-cell subset mainly distributed in mucosal surfaces. Two distinct lymphomas derived from these cells have been recognized: hepatosplenic γδ T-cell lymphoma (HSTL) and primary cutaneous γδ T-cell lymphoma (PCGD-TCL). However, whether other anatomic sites may also be involved and whether they represent a spectrum of the same disease is not well studied. The lack of TCRβ expression has been used to infer a γδ origin when other methods are not available. We studied 35 T-cell tumors suspected to be γδ TCL using monoclonal antibodies reactive with TCR δ or γ in paraffin sections. We were able to confirm γδ chain expression in 22 of 35 cases. We identified 8 PCGD-TCL, 6 HSTL, and 8 γδ TCL without hepatosplenic or cutaneous involvement involving mainly extranodal sites. Two such cases were classified as enteropathy associated T-cell lymphoma, type II. The other γδ TCL presented in the intestine, lung, tongue, orbit and lymph node. In addition we observed 13 cases with mainly extranodal involvement that lacked any TCR expression (“TCR silent”). In all cases an NK origin was excluded. In conclusion, the lack of TCRβ expression does not always predict a γδ T-cell derivation since TCR silent cases may be found. The recognition of γδ TCL presenting in extranodal sites other than skin and liver/spleen expands the clinical spectrum of these tumors. However, non- HSTL γδ TCL do not appear to represent a single entity. The relationship of these tumors to either HSTL or PCGD-TCL requires further study.

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Synchronous parathyroid and papillary thyroid carcinoma are extremely rare. To our knowledge, only 15 cases have been reported in the last four decades. We describe a 50-year-old female without significant past medical or family history and no previous trauma presented with left heel pain that prompted her to seek medical attention. Physical examination was notable for a painless nodule at the left thyroid lobe. Laboratory evaluation showed a serum calcium level of 14.3 mg/dL (8.6–10.3 mg/dL) and intact parathyroid hormone level of 1160 pg/mL (12–88 pg/mL). 99Tc-sestamibi dual-phase with single-photon emission computed tomography fused images showed increased uptake at the left-sided inferior parathyroid gland. Neck ultrasound showed a 1.4 cm heterogeneous nodule in the middle-third of the left thyroid gland and a solitary 1.9 cm vascularized and hypoechoic oval nodule that was considered likely to represent a parathyroid adenoma. Due to its clinical context (severe hypercalcemia and very high levels of PTH), parathyroid carcinoma (PC) was suspected although imaging studies were not characteristic. The patient underwent en bloc resection of the parathyroid mass and left thyroid lobe and central neck compartment dissection. Pathology analysis revealed classical papillary thyroid carcinoma of classical subtype and parathyroid carcinoma. Immunohistochemical staining was positive for cyclidin D1 and negative for parafibromin. High clinical suspicion is required for parathyroid carcinoma diagnosis in the presence of very high level of parathyroid hormone, marked hypercalcemia, and the existence of any thyroid nodule should be approached and the coexistence of other carcinomas should be considered.

Objective: Liposarcoma is the most common histotype of retroperitoneal sarcomas, representing up to 45% of all cases. We report a rare combination of acromegaly and liposarcoma in the same individual. Methods: Laboratory and imaging studies including an oral glucose tolerance test, measurements of growth hormone (GH) and insulin-like growth factor-1 (IGF-1), and a computed tomography scan were performed. Results: The patient was a 60-year-old male with a history of acromegaly diagnosed on the basis of elevated IGF-1 at 1,373 ng/mL (age-appropriate reference range is 87 to 225 ng/mL) and macroadenoma treated with transsphenoidal surgery. He presented 8 years later with a history of abdominal distension and weight loss. Physical examination was notable for a right-sided abdominal mass that was tense and non-fluctuant. Two years earlier, he had a post oral glucose tolerance test GH level <0.25 ng/mL and IGF-1 level of 256 ng/mL (age-appropriate reference range is 55 to 206 ng/mL). Pituitary magnetic resonance imaging reported a 3.7 × 2.0-mm left-sided parasagittal lesion. Computed tomography scan showed a 25.0 × 22.0 × 32.3-cm heterogeneous giant mass in the right abdomen corresponding to a liposarcoma causing displacement of kidney, liver, and bowel loops. The patient was treated with a complete en bloc resection of the liposarcoma with the right kidney (45 × 33 × 17 cm) and tumor (9,400 g). Immunohistochemical examination revealed positive IGF-1 and GH staining. The patient suffered postoperative gastrointestinal bleeding that resulted in hemorrhagic shock and died on the 29th postoperative day after a cardiorespiratory arrest. Conclusion: Acromegalic patients are at increased risk of developing various types of neoplasms. This is the first documented coexistence of liposarcoma and history of acromegaly.

Objective Inherited MAX gene pathogenic variants (PVs) increase risk for pheochromocytomas (PCCs) and/or paragangliomas (PGLs) in adults and children. There is little clinical experience with such mutations. This report highlights an important approach. Methods Clinical assessment, including blood chemistry, imaging studies, and genetic testing were performed. Results A 38-year-old Hispanic woman was diagnosed with PCC in 2015, treated with adrenalectomy and referred to endocrinology clinic. Notably, she presented to her primary care physician three years earlier complaining of left flank pain, intermittent diaphoresis and holocranial severe headache. We confirmed severe hypertension (180/100 mmHg) over multiple antihypertensive regimens. Biochemical and radiological studies work-up revealed high plasma metanephrine of 255 pg/mL (normal range &lt; 65), and plasma normetanephrine of 240 pg/mL (normal range &lt; 196). A non-contrast computed tomography scan of the abdomen revealed a 4.2 x 4.3 x 4.9 cm, round-shaped and heterogenous contrast enhancement of the left adrenal gland, and a 2-mm nonobstructive left kidney stone. A presumptive diagnosis of secondary hypertension was made. After pharmacological therapy, laparoscopic left adrenalectomy was performed and confirmed the diagnosis of pheochromocytoma. Based on both her age, family history and a high suspicion for genetic etiology, genetic testing was performed which revealed the presence of a novel likely pathogenic variant involving a splice consensus sequence in the MAX gene, designated c.64-2A&gt; G). Conclusion The phenotype of MAX PV-related disease and paraganglioma are highlighted. The novel c.64-2A&gt; G mutation is reported here and should be considered on the diagnostic work-up of similar cases.