Refractory pediatric nonrhabdomyosarcoma soft tissue sarcoma associated with ectopic production of beta hCG and hypercalcemia induced by PTHrP

Abstract

A 3-month-old male with a mass on the right side of his back was admitted to our hospital. The tumor was a pathologically high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). Treatment included subtotal tumor resection followed by chemotherapy. Elevation of serum beta hCG and hypercalcemia with detection of PTHrP was associated with tumor progression. The tumor was refractory to multiagent chemotherapy, and the patient died of the disease at 22 months of age. This case is novel in demonstrating a beta hCG secreting refractory NRSTS.

Ectopic Production of β-hCG by Osteosarcoma

“…2 Sporadic reports of this phenomenon exist for other types of sarcoma, including chrondrosarcoma, 3 leiomyosarcoma, 4-7 and unspecified nonrhabdomyosarcoma. 8 To the best of our knowledge, reports of b-hCG secretion in osteosarcoma are rare with only a few patients reported in the literature (Table 1). Boss et al 9 described a 57-year-old woman with elevated serum b-hCG discovered incidentally during screening for use of an investigational agent to treat relapsed metastatic osteosarcoma.…”

“…15 In addition, the finding of positive tumor and serum b-hCG was suggestive of osteosarcoma, although as noted above, this marker has been identified in other sarcomas, mainly nonrhabdomyosarcoma soft tissue sarcomas. [3][4][5][6][7][8] It was not until her tumor was resected that osteoid was identified and she was officially given the diagnosis of epithelioid osteosarcoma. This is not surprising since epithelioid osteosarcoma is typically associated with scant osteoid production, which may only be identified upon extensive sectioning of the resection in some cases.…”

“…1,2 Uncommonly, sarcomasincluding osteosarcoma-have been reported to express hCG with elevated levels of the b subunit (b-hCG) detectable in the serum. [3][4][5][6][7][8] Herein, we report a case of an adolescent female patient with a primary bone sarcoma who was incidentally discovered to have elevated serum b-hCG that served as a useful marker of treatment response. We report her clinical presentation and course and also review the available literature on the topic.…”

Dedifferentiated Liposarcoma With Rhabdomyosarcomatous Differentiation Producing HCG: A Case Report of a Diagnostic Pitfall

“…Of these, 6 were leiomyosarcomas, [17][18][19][20][21][22] 6 osteosarcomas, [23][24][25][26][27][28] 1 chondrosarcoma, 29 1 synovial sarcoma, 30 and 1 nonrhabdomyosarcoma soft tissue sarcoma in a pediatric patient. 31 In 2 recent studies, previously diagnosed osteosarcomas were retrospectively tested for HCG. A significant number of specimens (27 of 51 tumors in one study and 4 of 32 in the other) exhibited weak cytoplasmic HCG expression, and one specimen in each study was strongly positive for HCG.…”

Don’t cancel the surgery just yet! A case report of positive preoperative pregnancy test due to a soft tissue sarcoma production of ectopic beta human chorionic gonadotropin

“…It is even more rare for these metastases to express β-hCG, as this case did. Inoue et al 13 reported in 2011 a case of a nonrhabdomyosarcoma soft tissue sarcoma in a 3-month-old with a large back mass. Unfortunately, due to subtotal resection and chemotherapy resistance, the tumor progressed and this patient died of disease at 22 months old.…”